A PEER-REVIEWED FORUM FOR NURSE PRACTITIONERS
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Prisoner’s Visit to ED Prompts Lawsuit
An Underdiagnosed Cause of Resistant Hypertension
Preventing Shingles: Raising Awareness and Promoting Vaccination An estimated 1 million people develop herpes zoster each year in the US.
Director Nikki Kean email@example.com Medical editor Kristin Della Volpe
FROM THE DIRECTOR
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I have been watching the COVID-19 vaccination rates in the United States with some dismay. According to the Centers for Disease Control and Prevention (CDC), a little less than half of Americans are fully vaccinated (48.5%). This is well below the estimated 70% to 90% vaccination rate needed to achieve herd immunity. The state with the highest vaccination rate is Massachusetts, with 62.7% of its population fully vaccinated; the states with the lowest rates are Mississippi and Alabama, with 33% fully vaccinated. Since May 2021, 13.2% of adolescents aged 12 to 18 years have received at least 1 dose of the COVID-19 vaccine. Disappointedly, only 41.8% of young adults aged 18 to 24 years have been fully vaccinated. With colleges, high schools, and elementary schools set to open in the fall, the lack of herd immunity may contribute to the current wave of infections — especially with the rise in the Delta variant, which now accounts for 58% of COVID19 cases in the US (an increase from 3% in May). It is not just the COVID-19 vaccine that has been underutilized in America. This month’s cover article by Mary Jane S. Hanson, PhD, CRNP, FNP-BC, highlights the low vaccinations rates among older adults against shingles. Shingles vaccinations reached a peak in 2018, with 34.5% of adults older than 60 years vaccinated nationwide. Since the approval of the first shingles vaccine in 1995, more than 3.5 million cases of varicella and 100 deaths have been prevented each year. Also, it has been widely reported that pediatric and adolescent vaccination rates dropped at the beginning of the COVID-19. Many nurse practitioners and PAs have worked to provide alternative sites for vaccinations. Although vaccinations increased between June and September 2020, the increase did not achieve sufficient catch-up coverage, noted the CDC. To help in the safe reopening of schools, the CDC issued a call to action encouraging health care systems, health care providers, schools, parents, and state and local governments to work together to ensure that students are caught up on all routinely recommended vaccinations. Nikki Kean, Director The Clinical Advisor
8 THE CLINICAL ADVISOR • JULY/AUGUST 2021 • www.ClinicalAdvisor.com
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America, Roll Up Your Sleeves!
Assistant editor Jeanelle Jacobs
CONTENTS J U LY / A U G U S T 2 0 2 1
Conference Roundup ■ American Association of Nurse Practitioners ■ American Academy of PAs
FEATURES 20 Improving stroke diagnosis
1 Preventing Shingles: Raising Awareness and 2 Promoting Vaccination Shingle vaccination rates among older adults remain low. 7 Spinal Muscular Atrophy: Early Diagnosis for 2 Proactive Management The disorder targets motor neurons in the central nervous system. 5 Clinical Challenge: An Underdiagnosed Cause of 3 Resistant Hypertension A 30-year-old woman presents with headache, nausea, vomiting, diarrhea, and abdominal pain that started the previous day.
27 Progressive muscle atrophy and weakness
39 Autoimmune disease causing hair loss
47 Finger injury poorly managed in ED
From the Director America, Roll Up Your Sleeves!
Web Roundup A summary of our most recent opinion, news, and multimedia content from ClinicalAdvisor.com.
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CLINICAL CHALLENGE ClinicalAdvisor.com/CaseStudy
HBV Suppression Lowers Liver Cancer Rates in Patients Coinfected With HIV
Brady Pregerson, MD Traumatic Eye Injury A 22-year-old man is brought to the emergency department for the management of facial trauma sustained during an assault at a nightclub. The patient thinks he was knocked out but isn’t sure. He denies any vomiting, change in vision, or other complaints. See the full case at: ClinicalAdvisor.com/ Case_July_August21
Patients coinfected with HIV are less likely to develop liver cancer once chronic hepatitis B virus infection is suppressed to undetectable levels.
Teen Boys With High BMI Are at Risk for Moderate to Severe Sleep Apnea Adolescent boys with a higher BMI, adenotonsillar hypertrophy, and sleepdisordered breathing are at increased risk for developing moderate to severe obstructive sleep apnea.
Preeclampsia May Be a Risk Factor for Later-Life Stroke Women with a history of preeclampsia had a nearly 4-fold increased risk for later-life stroke compared with women without such history.
Frequency and Intensity of Tick-Borne Disease Increases in Midwest Climate change, population shift from urban to suburban areas, and increased population of white-tailed deer have contributed to the increase of tick-borne diseases throughout the Midwest.
MY PRACTICE ClinicalAdvisor.com/MyPractice Kristin Della Volpe Hospital Nurse-to-Patient Staffing Standards Bill Has Mixed Support Congress is considering a federal bill that would set minimum nurse-topatient ratios for every hospital unit and provide support for nursing best practices research, whistleblower protections, and education for nurses through loan repayment and scholarship programs. However, not all organizations support standardized nurse-to-patient ratios.
FDA: Systemic Side Effects Reported After Hand Sanitizer Application The FDA issued a safety communication about potential side effects when alcohol-based hand sanitizer is applied to the skin in enclosed spaces.
12 THE CLINICAL ADVISOR • JULY/AUGUST 2021 • www.ClinicalAdvisor.com
Natalie R. Baker, DNP, ANP-BC, GNP-BC, CNE, GS-C, FAANP Reducing Social Isolation Is Key to Successful Aging The GAPNA President-Elect discusses essential factors in successful aging, reducing loneliness in older adults in rural areas, and the benefits of a geriatric walking clinic for older patients with diabetes.
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EXCLUSIVE TO THE WEB AT
Advisor Dx Interact with your peers by viewing the images and offering your diagnosis and comments. To post your answer, obtain more clues, or view similar cases, visit ClinicalAdvisor.com/AdvisorDx. Learn more about diagnosing and treating these conditions, and see how you compare with your fellow colleagues. Check out some of our latest cases below!
Diffuse Rash Affecting Fingertips A 28-year-old woman presents with fever and diffuse, tender, erythematous to violaceous punctate lesions on her limbs, trunk, face, fingertips, and palms. She also has anemia, a positive Coombs test, and leukopenia. One year ago, the woman was diagnosed with systemic lupus erythematosus with cutaneous and renal involvement. CAN YOU DIAGNOSE THIS CASE?
• Subacute lupus erythematosus • Small-vessel vasculitis in systemic lupus erythematosus • Antiphospholipid syndrome • Urticarial vasculitis ● See the full case at ClinicalAdvisor.com/DermDx_July_August21
In partnership with
Journal of Orthopedics for Physician Assistants
Hip Tightness After Total Hip Replacement A 76-year-old man presents with right hip “tightness” that has progressed over the past 4 months. He denies significant pain in the hip and his gait remains unchanged. He underwent a hip replacement 1 year prior. The patient’s right hip internal rotation measures 15°; abduction, 20°; and hip flexion, 95°. Anteroposterior radiograph of the pelvis shows heterotopic ossification in the right hip. WHAT IS THE BEST TREATMENT OPTION FOR THIS PATIENT?
• Observation • Indomethacin for 6 weeks
• External beam radiation • Surgical excision
● See the full case at ClinicalAdvisor.com/OrthoDx_July_August21
www.ClinicalAdvisor.com • THE CLINICAL ADVISOR • JULY/AUGUST 2021 13
Conference Roundup Highlights From AANP and AAPA 2021 Virtual Spring Meetings
AAPA ADOPTS PHYSICIAN ASSOCIATE AS OFFICIAL TITLE FOR PROFESSION The American Academy of PAs (AAPA) House of Delegates passed a resolution to affirm Physician Associate as the official title of the PA profession. A majority of delegates voted for the name change (198 to 68) during the AAPA 2021 conference. The PA profession was created more than 50 years ago. During that time, there have been several efforts to have the American Academy of PAs endorse a different title. Many PAs have long disliked the word “assistant,” often feeling that this misrepresents the profession and the work PAs do. “Changing our title from assistant to associate is going back to our PA roots. PAs have had many titles throughout the years, one of which is physician associate,” noted Melinda Moore Gottschalk, MPAS, PA-C, DFAAPA, from the University of Mary Hardin-Baylor PA Program. “PAs in some universities are still graduated as a physician associate.” “I’ve long supported the name of Physician Associate for our profession. I’ll admit my bias up front. I graduated from the Yale Physician Associate Program over 22 years ago, which has been the name since the Yale PA Program’s inception,” Jonathan Weber, MA, PA-C, said in an interview with Clinical Advisor. In a letter to its members, AAPA acknowledged that this is a complicated
issue. “Title change implementation is a complex and intricately interwoven undertaking requiring a thoughtful and well-timed strategy involving a variety of stakeholders — not only other national PA organizations (PAEA, NCCPA, and ARC-PA), PA programs, and AAPA constituent organizations, but also state and federal governments, regulators, and employers,” the AAPA wrote.
DO PAs NEED A DOCTORAL DEGREE? Do PAs need a doctoral degree? It depends. For persons in academia and particularly individuals seeking higher leadership positions, a doctoral credential may be valuable. However, for PAs in clinical practice, the value of a doctoral
Having a doctoral degree is financially beneficial for PA faculty with leadership roles.
14 THE CLINICAL ADVISOR • JULY/AUGUST 2021 • www.ClinicalAdvisor.com
credential needs further investigation, according to research presented at AAPA 2021. Gerald Kayingo, PhD, MBA, PA-C, from the PA Leadership and Learning Academy, and colleagues studied job advertisements posted between 2014 and 2020. Of the 612 unique job postings from 232 PA programs, 50% of employers preferred or required a doctoral degree for leadership positions such as chair or program director.That number decreased to 36% for regular faculty positions. However, only approximately 24% of PA faculty and 45% to 48% of program directors reported having a doctoral degree, most commonly a Doctor of Philosophy. “Thirty-six percent in the job market are saying, ‘give us a candidate with a doctoral degree.’ However, only 27% have appropriate credentials,” Dr Kayingo explained. A wage gap analysis revealed the following: • Median income is $105,000 for a certified PA in clinical practice, $97,000 for certified PA faculty, and $103,000 for PA faculty with a doctoral degree. • A certified PA program director earns a median of $131,000; that number increases to $140,000 with a doctorate. “A doctorate degree made a huge difference if that candidate ended up assuming a leadership role,” Dr Kayingo said. However, for regular faculty, the doctorate did not seem to provide a financial benefit. Continues on page 18
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■ American Association of Nurse Practitioners ■ American Academy of PAs
LEFT HAND PAGE / P4
Education Association (PAEA), and the
salaries; among PA faculty, women earned the n/a data was adjusted bp for factors such AAPA 07/07/21 as experience, specialty, leadership roles, PMSxxxx 6:08 am the salary for and weekly hours worked, women faculty members rose to 94% of their male counterparts. The disparity was slightly less for full-time PA faculty than for all PAs. To reduce the inequity, the researchers recommended these approaches: • Perform regular pay equity analyses to ensure that salaries among all PAs remain based on relevant variables, such as education, length of employment, positions, and contributions to the institution or clinic • Determine compensation in a group setting and make an overall effort to increase transparency pertaining to salary • Offer salary negotiation education and advocacy training for practicing PAs and PA students.
M49431_PP-US-REL-CT-2100114_Myfembree_BriefSummary_BW_CommonA_size_Mv04_ClinicalAdvisor_LEFT_PG4 Continued from page 14 National Certification Commission of 91% of men’s compensation.When
Giant Creative Strategy 49431 Physician Assistants (NCCPA).
GENDER WAGE GAP PERSISTS FOR PAs Gender pay disparities in the PA profession have been well-documented for decades.Although the gap is closing in the wake of social reckonings and advocacy for women’s rights, the gap persists, according to results presented at AAPA 2021. “While PAs are doing better in comparison with non-PA women, there is still a wage gap,” said coauthor Amy E. Baker, MS, PA-C, program director for the Physician Assistant Program at West Chester University. Baker and her colleagues found that women who are PAs earn approximately 90% of their male counterparts’ salaries.This compares with women nationally, who earn on average 82 cents for every dollar a man makes. The findings are based on data collected by the AAPA, Physician Assistant
In the PAEA 2017 Faculty and PMSxxxx Directors Survey,PMSxxxx the meanPMSxxxx salary among men was $9432 higher than that among women ($102,638 vs $93,206, respectively). Nationwide data from the NCCPA also indicated a significant gender salary disparity. Data from the 2018 AAPA Salary Report, in which salary information was cross referenced with various employment factors (eg, average number of hours worked per week and on-call duties) showed that the mean salary among men was $11,500 higher than that among women ($112,500 vs $101,000, respectively). Although clinically practicing PAs earn higher salaries than academics, the gender pay disparity is greater for those in clinical practice. Among PAs in clinical practice, women earned 83% of men’s
18 THE CLINICAL ADVISOR • JULY/AUGUST 2021 • www.ClinicalAdvisor.com
NP-LED CALLS WITH THE CHRONICALLY ILL REDUCE ED VISITS For chronically ill, home-based patients, telephone access to nurse practitioners (NPs) is associated with decreased emergency department (ED) visits, according to findings presented at AANP 2021. The research was part of an academic-clinical partnership between the University of Rhode Island College of Nursing and local federally qualified health centers. Through the initiative, Denise Coppa, PhD, FNP-C, FAANP, FAAN, and colleagues studied the value of telephone encounters in patients with chronic conditions including diabetes, congestive heart failure, COPD, asthma, hypertension, and psychiatric illnesses who had 3 or more ED visits or hospitalizations within the previous 6 months. Electronic medical record data were collected between April 15, 2016, and June 30, 2018, and compared with 2 periods before initiation of the homebased primary care program. The cohort included 157 patients; 98% had difficulty accessing health care, 54% were on Medicare, 21% were on Medicaid, and 1% were uninsured. Although patients with 4 or more telephone encounters had an increased number of ED visits during the home care intervention, these patients were likely the most medically compromised, the authors noted. This increase was offset by a decrease in ED visits among patients with 1 to 3 telephone encounters. The overall number of ED visits was decreased by 13% (P =.03) with each 1-unit increase in the number of telephone encounters for patients with hypertension, diabetes, heart disease, dyslipidemia, and arthritis, the researchers reported. “Providing NPs with a cost-effective, easy-to-use, telephone model to decrease recidivism provided positive outcomes for both the patient and the
health care system. Medical homes and appropriate follow-up are an evidencebased approach to providing the support patients need to decrease repeated ED visits,” commented AANP 2021 attendee Mary Koslap-Petroco, DNP, PPCNPBC, CPNP, FAANP, of Stony Brook University School of Nursing in Stony Brook, New York. “Federal support for academic-community partnerships imports potential for improved patient outcomes, decreased ED visits and hospitalizations, and health care costs,” the researchers concluded. Telephone encounters by NPs with home-based primary care “patients are necessary and worthwhile.”
PAIN MANAGEMENT EDUCATION FOR APRNs IS STILL LACKING Many early-career advanced practice register nurses (APRNs) report low confidence and competence in managing chronic and acute pain, according to research presented at AANP 2021. Data from the 2020 AANP Member Educational Needs assessment survey showed that 1 in 5 of the more than 6000 respondents would like additional continuing education on pain management. Pain management also ranked eighth on a list of areas in which NPs requested additional resources. To assess the current state of pain education for APRNs, the researchers conducted a nationwide survey of APRNs. Overall, 69% of APRNs reported receiving less than 5 hours of pain management education during primary training; 24% reported receiving 5 to 10 hours of pain education; 5% received more than 10 hours; and 2% did not recall or did not respond to this question. Most reported that on-the-job training served as their primary mode of pain management education followed by self-directed continuing education activities.
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Most APRNs received less than 5 hours of pain management education in training.
Fifty-two percent of respondents (n=144) reported spending at least 50% of their time managing pain in daily practice. Of those respondents, 78 worked in either pain management or palliative care specialty whereas the remaining worked in family or internal medicine, among other specialties. Respondents in primary care, internal medicine, or surgery reported spending less time on pain management practices compared with APRNs in other specialties. When asked about their level of preparation for caring for patients with basic chronic or acute pain management needs, 67% of participants felt neutral to unprepared to do so during their first year of clinical practice. These results are indicative of “low confidence and perceived competence in providing pain management across acute and chronic pain settings,” the researchers noted. “For those who focus their careers in pain management as a specialty, a system for certification would help to facilitate a metric of quality control and further delineation of competency in practice,” they concluded.
www.ClinicalAdvisor.com • THE CLINICAL ADVISOR • JULY/AUGUST 2021 19
OPIOID GUIDELINE RESOURCES FOR NURSE PRACTITIONERS Four resources to support NPs in implementing opioid guidelines were highlighted by the CDC’s Loretta Jackson Brown, PhD, RN, CNN, in a poster session at AANP 2021. The CDC provides 2 free, interactive, online training modules for health care providers, including NPs: 1. A Nurse’s Call to Action for Safer Opioid Prescribing Practices 2. Using the Prescr iption Drug Monitoring Program to Promote Patient Safety in Opioid Prescribing and Dispensing The third tool is the CDC’s Quality Improvement and Care Coordination handbook, and the fourth is the CDC Opioid Guideline App for use during patient interactions.The free app includes a morphine milligram equivalents calculator, an interactive interviewing feature, and summary of key recommendations. Dr Brown highlighted the following 3 main focus areas of the CDC Guideline for Prescribing Opioids for Chronic Pain: 1. Determine when to initiate or continue opioids for chronic pain 2. Determine opioid selection, dosage, duration, follow-up, and discontinuation 3. Assess risk for misuse and addressing harms of opioid use Nurse practitioners should educate patients on all risks, potential side effects, and interactions associated with opioid therapies and be familiar with nonopioid and nonpharmacologic alternatives for chronic pain management. Long-acting or extended-release formulations should be reserved for patients with severe, continuous pain, Dr Brown explained. The CDC recommends identifying potential drug interactions, such as benzodiazepines, that may increase the risk for overdose. “By implementing the CDC guideline recommendations, NPs will improve the safety and effectiveness of pain treatment
for their patients by reducing the risk associated with long-term opioid therapy,” Dr Brown noted.
GOING BEYOND FAST WHEN DIAGNOSING ACUTE STROKE Stroke diagnosis can be challenging for clinicians, primarily because the clinical manifestations, patient-reported symptoms, and physical examination findings are highly variable. Subtle stroke signs are frequently missed, researchers reported at AANP 2021. The FAST acronym — face, arm, speech, and time — is widely used to identify stroke symptoms, but the tool can exclude more subtle signs and symptoms.To describe the limitations of currently available stroke screening as well as newer modifications made to these tools to improve diagnostic accuracy, Stephanie Rosser, DNP, APRN, of The University of Texas Medical Branch School of Nursing in Galveston, Texas, conducted a literature review. Twenty-four articles were reviewed and 8 were included in the final analysis: 4 systematic reviews and 4 case-control, cohort, or descriptive studies.
The ROSIER scale, BE-FAST, and CPSS may have improved accuracy in stroke diagnosis.
20 THE CLINICAL ADVISOR • JULY/AUGUST 2021 • www.ClinicalAdvisor.com
In the first systematic review, the Melbourne Ambulance Stroke Scale (MASS), Medic Prehospital Assessment for Code Stroke (MedPACS), and Ontario Prehospital Stroke Screening (OPSS) tools were evaluated only in a single prehospital setting, with high variability in the sensitivity and specificity of the studies.The remaining 3 systematic reviews, found that the Cincinnati PreHospital Stroke Scale (CPSS) had a high degree of both sensitivity and specificity in field settings and should be used preferentially over other scales. Zhelev et al found that both Recognition of Stroke in the Emergency Room (ROSIER) and FAST demonstrated similar levels of accuracy in ED settings. However, the ROSIER scale was evaluated in more studies with “consistently higher sensitivity” compared with FAST, making it the test of choice. Meyran and colleagues reported that both FAST and OPSS were associated with “a positive increase in the number of patients who received timely reperfusion treatment.” The case-control, cohort, and descriptive studies found that patients who presented with typical posterior stroke symptoms (nausea, vomiting, and dizziness) received delayed evaluation and 14% of stroke diagnoses were missed using the FAST acronym. However, adding balance and eye examination to the acronym (BE-FAST) reduced the number of missed strokes. Results of a study by Oostema and colleagues showed that adding the finger-to-nose test to the CPSS improved recognition of posterior stroke in field settings, Dr Rosser explained. Current stroke screening tools generally perform well when patients display overt signs of stroke such as hemiparesis and neglect, but lack sensitivity in recognizing more subtle signs associated with posterior circulating stroke, Dr Rosser concluded. ■
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FEATURE: MARY JANE S. HANSON, PhD, CRNP, FNP-BC, FAANP, FAAN
Preventing Shingles: Raising Awareness and Promoting Vaccination Despite availability of a highly effective shingles vaccine and almost universal risk among those born before 1980, Americans are not getting vaccinated.
© BSIP / WITT-DEGUILLAUME / MEDICALIMAGES.COM
The US falls short in immunizing adults against herpes zoster.
n estimated 1 million people develop herpes zoster (HZ), or shingles, each year in the United States, and approximately 1 in 3 Americans will develop HZ in their lifetime.1 HZ occurs because of reactivation of the varicella-zoster virus, which causes varicella (chickenpox) and remains dormant in the dorsal root ganglia.2 When the HZ virus becomes reactivated later in life, patients develop a painful, maculopapular rash that matures into vesicles and commonly appears along 1 or 2 adjacent dermatomes along the face and/or torso. The rash usually does not cross the body’s midline. Before blisters appear, patients may feel pain, tingling, and/or itching along the nerve endings infected by the virus. The blisters begin to scab over in 7 to 10 days and clear up completely within 2 to 4 weeks.1,2 Any patient who has had varicella can develop HZ and the risk increases with age, especially in patients older than 50 years. Conditions that may compromise the immune system, such as HIV, lymphoma, and leukemia, also raise the risk for HZ. Treatments that suppress the immune system, such as radiation, chemotherapy, or steroids, increase an individual’s risk for shingles.2 Although some patients may not remember having chickenpox, more than 99% of Americans born before 1980 have had it.1 In the early 1990s, an average of 4 million people developed varicella and 100 to 150 died of the disease every year.1 Since the first varicella vaccine became available www.ClinicalAdvisor.com • THE CLINICAL ADVISOR • JULY/AUGUST 2021 21
INFECTIOUS DISEASE: HERPES ZOSTER
RZV is recommended for the prevention of HZ and related complications for immunocompetent adults previously administered ZVL. in the US in 1995, more than 3.5 million cases of varicella, 9000 hospitalizations, and 100 deaths have been prevented each year by varicella vaccination.1 Children who have been vaccinated against varicella have a lower risk for HZ compared with children who became infected with the varicella virus.1,2 The most common complication of HZ is postherpetic neuralgia (PHN), which occurs in 10% to 18% of those affected. The chronic nerve pain lasts after the blisters have healed.The course of PHN can be severe and life-changing, lasting months to years.3 Early treatment of HZ can reduce the severity and incidence of PHN. Less common but severe complications of HZ include blindness caused by secondary bacterial infection, pneumonia, and hearing problems.1-3 Treatment of Shingles
Treatment of shingles includes antiviral medications, which reduce both the acute pain of herpes zoster and risk for PHN. Antiviral treatment should be started as soon as possible and is most effective when started within 72 hours of rash onset (Table).4
for adults 50 years and older and is preferred over zoster vaccine live (ZVL, Zostavax), which was approved in 2006 and removed from the market in November 2020 because of the superior efficacy of RZV.6,7 RZV is recommended for the prevention of HZ and related complications for immunocompetent adults who previously received ZVL.6 The vaccination consists of 2 doses (0.5 mL each) administered intramuscularly 2 to 6 months apart.6,7 Two doses of RZV are more than 90% effective at preventing HZ and PHN. The vaccine’s effectiveness against HZ remains greater than 85% for at least the first 4 years after vaccination.2 The ACIP also recommended that people with a history of HZ should be vaccinated because HZ can recur. Individuals with a current outbreak of HZ should wait until the acute phase of the disease is over and all lesions have completely healed before getting vaccinated. It is not necessary to screen for a history of varicella before administering the shingles vaccine. However, an individual who presents with negative laboratory evidence of chickenpox probably would not benefit from the vaccine because it does not protect against chickenpox infection.7
Shingles Prevention During COVID-19
According to the Centers for Disease Control and Prevention (CDC), HZ vaccination is an essential preventive care service for older adults that should not be delayed or discontinued during the COVID-19 pandemic unless a patient is suspected or confirmed to have COVID-19.5 Only 1 Food and Drug Administration (FDA)-approved vaccine for the prevention of HZ is available: recombinant zoster vaccine (RZV, Shingrix). According to the Advisory Committee on Immunization Practices (ACIP), RZV is recommended for the prevention of HZ and related complications TABLE. Antiviral Therapies for Herpes Zoster in Adults4 Name
Length of Treatment
800 mg 5 times a day
500 mg 3 times a day
1000 mg 3 times a day
All dosages are for oral administration.
Safety and Side Effects
Postlicensure surveillance data on RZV from October 2017 through June 2018 reaffirm that the vaccine is safe and highly effective. Serious adverse events were rare. However, as was seen in clinical trials, local and systemic reactions can occur including pain, swelling, and redness at the injection site as well as fever, chills, and body aches.7 Counseling patients about potential side effects is especially important during the COVID-19 pandemic because some side effects of shingles vaccination may be similar to symptoms of COVID-19.5 These reactions are self-limited and resolve in a few days.8 Because the shingles vaccine stimulates the immune system, patients can expect such reactions to occur. The immune system responses are not a sign of allergy to the vaccination, and patients should not omit the second dose if such reactions occur, especially because the effectiveness of a single dose of RZV has not been studied.7 Reactions to the first dose of the vaccine have not been shown to predict reactions to the second dose. The only contraindication to RZV is a severe allergic reaction to components of the vaccine. Adults with chronic medical conditions including diabetes mellitus, chronic pulmonary disease, and chronic renal failure should receive RZV. The vaccine also is indicated for immunocompromised persons,
22 THE CLINICAL ADVISOR • JULY/AUGUST 2021 • www.ClinicalAdvisor.com
Shingles vaccination rates among adults 60 years and older remain low and increased from 6.7% in 2008 to only 34.5% in 2018. including those taking immunosuppressive therapy or recovering from an immunocompromising illness. However, RZV’s effectiveness in these individuals has not been studied.7 Vaccination Rates
The United States is falling short in immunizing older adults against HZ. Findings from the National Health Interview Survey revealed that shingles vaccination rates among adults 60 years and older increased from 6.7% in 2008 to 33.4% in 2016 and then remained relatively constant through 2018, reaching 34.5%.9 Shingles vaccination rates did not differ by sex in 2018. However, non-Hispanic White adults (38.6%) were approximately twice as likely as non-Hispanic Black (18.8%) and Hispanic (19.5%) adults to have ever received a shingles vaccination.9 Vaccination coverage was highest for those with higher incomes and those who had more than a high school education. For example, adults with incomes below the federal poverty level ($12,760 for an individual) were only half as likely to get immunized as adults with annual incomes of more than $25,000.9 These findings are consistent with disparities seen with other adult vaccines.10 The cost of RZV can have a negative impact on vaccination rates, especially among patients of low socioeconomic status, many of whom do not have health insurance. Commercial health insurance plans generally cover RZV when it is given at
POLL POSITION As of 2018, what percentage of US adults aged ≥60 years were vaccinated against herpes zoster? 1% 7.58% ■ 22.8% ■ 34.5% ■ 43.8%
a provider’s office. However, RZV is covered under Medicare Part D plans, unlike most other vaccinations, including influenza and pneumococcal, which are covered under Part B. Part D vaccinations typically are administered at a retail site of care, such as a pharmacy, and the out-of-pocket cost to the patient can vary greatly because of different plan designs. Medicaid may or may not cover the vaccine.11 To improve shingles vaccination rates, the CDC encourages health care providers to increase awareness among patients of the severity of HZ and efficacy of the RZV as well as use of tools for implementing the Standards for Adult Immunization Practice.10,12 To address racial and ethnic disparities in vaccination rates, providers should include regular assessment of adult vaccination needs and offer to administer vaccinations as part of routine clinical care. In addition, the CDC suggests that providers consider developing immunization quality improvement projects at their practices to increase adult immunization rates.10,12 Information for Providers
Health care providers who previously had used ZVL should be aware of the differences in administration and storage for RZV. For example, RZV is supplied as 2 components: a single-dose vial of lyophilized varicella virus glycoprotein and a single-dose vial of adjuvant suspension. Both the antigen and adjuvant should be kept refrigerated between 2 ºC and 8 ºC (36 ºF and 46 ºF). If the components are frozen, they should be discarded. The vaccine should be administered immediately after being reconstituted or refrigerated and used within 6 hours. If it is not used within 6 hours after reconstitution, it should be discarded. Recombinant zoster vaccine must be given by intramuscular injection, unlike ZVL, which was given by subcutaneous injection. Two doses of RZV are required with the second dose given 2 to 6 months after the first dose.13 The vaccine series does not have to be restarted if more than 6 months have elapsed since the first dose, but the efficacy of alternate dosing regimens has not been studied.7 More information about mRNA COVID-19 vaccine coadministration with other vaccines is available at: https://www. cdc.gov/vaccines/covid-19/clinical-considerations/index.html Summary
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Greater efforts are needed by all health care providers to increase shingles vaccination rates among older adults. The Continues on page 26
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INFECTIOUS DISEASE: HERPES ZOSTER
vaccine has been shown to reduce the risk for shingles and PHN by more than 90% in people 50 years and older. During patient counseling, providers should emphasize the painful disease process, complications, and possible long-term sequelae of HZ as well as the proven safety and efficacy of RZV. Targeted efforts are needed to increase vaccination rates among older adults of low socioeconomic status and racial/ ethnic minorities. ■
6. Harpaz R, Ortega-Sanchez IR, Seward JF; Advisory Committee on Immunization Practices (ACIP). Prevention of herpes zoster: recommendations of the Advisory Committee on Immunization Practices (ACIP). MMWR Morb Mortal Wkly Rep. 2008;57(RR-5):1-30. 7. Dooling KL, Guo A, Patel M, et al. Recommendations of the Advisory Committee on Immunization Practices for use of herpes zoster vaccines. MMWR Morb Mortal Wkly Rep. 2018;67(3):103-108. 8. Hesse EM, Shimabukuro TT, Su JR, et al. Postlicensure safety surveillance of recombinant zoster vaccine (Shingrix) — United States, October 2017–
Mary Jane S. Hanson, PhD, CRNP, FNP-BC, FAANP, FAAN, is professor and director of the graduate nursing program at the University of Scranton and works at Lehigh Valley Physician Group – Family Medicine in Lehighton, Pennsylvania.
June 2018. MMWR Morb Mortal Wkly Rep. 2019;68(4)91-94. 9. Terlizzi EP, Black LI. Shingles vaccination among adults aged 60 and over: United States, 2018. NCHS Data Brief, no 370. Hyattsville, MD: National Center for Health Statistics. 2020. 10. Centers for Disease Control and Prevention. Vaccination coverage among
adults in the United States, National Health Interview Survey, 2016. Updated
1. Centers for Disease Control and Prevention. Shingles (Herpes zoster).
February 8, 2018. Accessed June 16, 2021. https://www.cdc.gov/vaccines/imz-
Accessed April 28, 2021. https://www.cdc.gov/shingles/
2. Centers for Disease Control and Prevention. Shingles (Herpes zoster):
11. Centers for Disease Control and Prevention. Shingles vaccination.
clinical overview. Accessed June 17, 2021. https://www.cdc.gov/shingles/
Updated January 25, 2018. Accessed June 16, 2021. https://www.cdc.gov/
3. Centers for Disease Control and Prevention. Shingles (Herpes zoster)
12. Centers for Disease Control and Prevention. Standards for
complications of shingles. Accessed April 28, 2021. https://www.cdc.gov/
adult immunization practice. Updated May 2, 2016. Accessed June 16, 2021.
4. Wareham DW, Breuer J. Herpes zoster. BMJ. 2007;334(7605):1211-1215.
13. Shimabukuro TT, Miller ER, Strikas RA, et al. Notes from the field:
5. Centers for Disease Control and Prevention. Frequently asked questions
vaccine administration errors involving recombinant zoster vaccine –
about Shingrix. Accessed June 16, 2021, https://www.cdc.gov/vaccines/vpd/
United States, 2017–2018. MMWR Morb Mortal Wkly Rep. 2018;67(20):
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26 THE CLINICAL ADVISOR • JULY/AUGUST 2021 • www.ClinicalAdvisor.com
FEATURE: MELLISA HALL, DNP, AGPCNP-BC, FNP-BC; JENNIFER TITZER EVANS, DNP, RN, NC-BC
Spinal Muscular Atrophy: Early Diagnosis for Proactive Management Early diagnosis and treatment of SMA can limit disease progression in children and adults, extending life expectancy and improving QOL.
© BOB TAPPER / MEDICALIMAGES.COM
SMA targets motor neurons in the CNS, resulting in progressive muscle atrophy, weakness, and paralysis.
pinal muscular atrophy (SMA) is a severe genetic condition that targets motor neurons in the central nervous system (CNS), resulting in progressive muscle atrophy, weakness, and paralysis.1,2 The condition affects 1 in 11,000 births but often eludes diagnosis based on clinical assessment findings.1 The availability of new treatments for SMA has changed the natural history of this disease. Early diagnosis and treatment of SMA can limit disease progression in children and adults, extending life expectancy and improving quality of life (QOL). SMA is categorized into 5 types according to age at onset and maximum motor function achieved (Table 1).1,2 Type 0, the most severe form of SMA, begins in utero; after birth, babies have difficulty moving, swallowing, and breathing and survive days to months if they are not treated.2 Type 1, the most common form, is diagnosed during infancy and associated with the inability to sit unsupported by the expected developmental stage (age, 9 months).1,2 Type 2 includes children who can sit unsupported but are unable to walk.1,2 Type 3 includes patients who can walk during childhood but have weak motor function, and type 4 begins in adulthood in patients with no previously identified neurologic motor deficits.1,2 Each type of SMA is associated with symmetric muscle weakness in the periphery with the more noticeable weakness in the lower extremities initially.1,2 Cognitive function is not affected in any type of this disease. The disease is a leading
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SPINAL MUSCULAR ATROPHY
TABLE 1. Spinal Muscular Atrophy Symptoms by Type1,2 Type
Age of Onset
• Decreased fetal movements • Severe weakness and neonatal hypotonia • Areflexia • Respiratory failure at birth • Facial diplegia • Atrial septal defects
1 (Infantile SMA or Werdnig-Hoffmann disease)
• Motor deficits, including loss of head control • Variable suck and swallow difficulties • Mild joint contractures • Normal or minimal facial weakness • Rapid disease progression leading to respiratory failure
2 (Intermediate form or Dubowitz disease)
• Weakness affects the lower extremities more than upper • Independent standing/walking is likely not achieved • Decreased or absent deep tendon reflexes • Finger tremor
3 (Juvenile form or Kugelberg-Welander disease)
• Can walk independently • Symptoms present as proximal muscle weakness (eg, falling and trouble climbing stairs)
4 (Late onset)
• Onset not clearly defined; often age 20-40 years • Motor milestones all achieved before proximal muscle weakness develops • Lifespan is not reduced
cause of infant mortality because of a single gene but longevity typically is not affected in SMA types 3 and 4.2-4 Epidemiology
This disorder is most commonly caused by genetic deletions or mutations in the survival motor neuron 1 (SMN1) gene, causing it to produce low levels of SMN protein. The inheritance pattern is autosomal recessive, and carrier frequency ranges from 1 in 45 to 1 in 100 people.2 Asian ethnicity is associated with the highest carrier frequency (2.4%).1 Because of the high mortality of SMA types 0 and 1, as well as the QOL implications of all types of SMA, carrier screening should be considered across ethnicities.4
SMA type 2 has similar clinical diagnostic clues, but bulbar symptoms and respiratory concerns are not as profound as in SMA type 1.2 Infants fail to reach motor development milestones of pulling up and walking by age 15 months.2 In types 3 and 4, patient and parental history is key to diagnosis.2 Because patients can walk and function as expected until adolescence or adulthood, clinical symptoms of muscle weakening often are ignored or missed, leading to delayed diagnosis (see Delayed Diagnosis of SMA Type 3:A Case Study).2 Clinicians should be aware of the genetic prevalence of SMA and consider the condition in the differential diagnosis of new-onset muscular weakness (Table 2, page 32).2 Screening and Diagnosis of SMA
Clinical Presentation of SMA Types
In SMA type 1, infants exhibit hypotonia of the extremities but demonstrate normal facial expressions.2 Cries often are weak and bulbar symptoms are present, leading to progressive feeding problems and aspiration.2 Infants with SMA type 1 presenting late in the disease process exhibit signs of respiratory distress and failure.2 Respiratory distress may be the presenting symptom in infants not seen for routine wellness exams.2
In 2018, SMA genetic screening was added to the Recommended Uniform Screening Panel for newborns in the United States.5,6 In addition, more states are adding SMA to their newborn screening panel. A positive newborn screening result allows for immediate confirmatory genetic testing and treatment to prevent SMA disease symptoms from starting or progressing.
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Continues on page 32
SPINAL MUSCULAR ATROPHY
TABLE 2. Differential Diagnosis of New-Onset Muscle Weakness in Children and Adults2 Duchenne and Becker muscular dystrophy
• Most often identified in early childhood (3-4 y)
Limb-girdle muscular atrophy
• Weakness of the shoulder or pelvic regions
• Ocular symptoms of ptosis or diplopia is the most common clinical finding (50% of patients) • Onset often in second or third decade
• Bilateral ascending motor paralysis • Often preceded by an infection
Amyotrophic lateral sclerosis
• Asymmetric muscle weakness of extremities is the most common clinical presentation • Next most frequent clinical symptom is bulbar weakness
Spinal and bulbar muscular atrophy
• Onset from age 20-60 y, with slow progressive weakness • Associated with endocrine dysfunction, often involving androgen resistance
For all patients, genetic testing for both the SMN1 and SMN2 genes is recommended to confirm the diagnosis.1,2,7 This testing should be be offered to patients presenting with SMA symptoms to allow for early intervention with newly developed pharmacotherapeutics that can slow or stop disease progression.7 Previously, electromyography and muscle biopsy were required to diagnose SMA but genetic testing now is considered the gold standard for diagnosis.2 Additional laboratory tests, including creatinine kinase levels, often are within normal range.1 For adult-onset SMA (type 4), history is key to the diagnosis and may include muscle fatigue not previously experienced, difficulty with long flights of stairs, and changes in walking/
Medication: • Nusinersen • Onasemnogene • Risdiplam
Physical/ occupational therapy
Nutrition/ speech therapy
FIGURE. Multidisciplinary care plan for spinal muscular atrophy.10
running stride and coordination. Patient history should be thorough to uncover any alterations in activity due to occupational changes or new hobbies/exercise habits. Patients should be queried about changes in functional abilities with each encounter. Functional assessment instruments help standardize evaluations between examiners. Examples of motor function assessment screening tools are the Timed Up and Go Test and the Assessment of Motor Function.8,9 Physical examination findings in older children and adults may include bilateral diminished deep tendon reflexes in the lower extremities. Reflexes should be quantified on a 0 to 4+ scale with each patient encounter. Strength testing of the extremities should be quantified on a 0 to 5+ scale and monitored in serial assessments.9 This ongoing clinical assessment helps evaluate peak muscle function, which is a predictor of disease severity and life expectancy. Pharmacologic Treatment
Management of SMA requires a multidisciplinary approach led by a pediatric neurologist (Figure).1 Given the various types of SMA and multiple clinical manifestations, treatment is individualized based on each patient’s functioning.10 Pharmacologic management of SMA became a reality in December 2016 with the approval of the first SMN-enhancing therapy, nusinersen, for all SMA types in patients of all ages.11 Since then, 2 other SMN-enhancing therapies have been approved: onasemnogene abeparvovec-xioi for patients up to 2 years of age and risdiplam for patients 2 months and older.12,13 Nusinersen Nusinersen binds with SMN2 genes to increase SMN protein production.11 The drug is administered intrathecally in
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4 loading doses, delivered every 2 weeks for 3 doses and then after 30 days for the fourth dose. Maintenance treatment should be given every 4 months for life.11 Nusinersen is associated with an increase in the achievement of motor milestones and motor function for patients with SMA type 1. In patients with SMA types 2 and 3, this treatment has been associated with improvements in or maintenance of motor function.11 Onasemnogene abeparvovec-xioi Gene therapy with onasemnogene abeparvovec-xioi is approved for the treatment of SMA in infants and children younger than 2 years.12 The treatment replaces the defective or missing SMN1 gene with a working copy and is given as a single intravenous infusion.12 Gene therapy works best when given early in the disease course, can be administered before symptom onset, and increases survival rates without permanent ventilation and the ability to sit unsupported in clinical trials.12 Risdiplam The newest agent, risdiplam, is approved for patients 2 months and older.13 This SMN2 pre‐mRNA splicing modifier increases SMN production and distribution in the CNS and peripheral tissues.13 Risdiplam is administered orally once daily.13 In clinical trials, risdiplam was linked to an increased rate of survival without permanent ventilation and ability to sit unsupported in infants and improved motor function in adults.
The SMA treatment team also includes rehabilitation professionals such as respiratory, physical, occupational, and speech therapists. Rehabilitation treatment involves care related to positioning, mobility, activities of daily living, stretching, and use of adaptive equipment to maximize functioning and QOL.1 Respiratory therapists are key health care professionals involved in the care of patients with SMA.10 Respiratory assessments should include pulse oximetry and capnography, along with an assessment of cough effectiveness. Also, patients with SMA types 1 and 2 often require airway support, including noninvasive positive pressure ventilation, continuous positive airway pressure, or tracheotomy ventilation. Parents of children with these airway support devices will require extensive education. Part of respiratory management for this patient population includes chest physiotherapy and mechanical cough assistance. Patients with SMA type 3 may have a higher risk for complications during acute illness and should be monitored closely for any signs of respiratory impairment.10 Because of the high risk for respiratory complications, patients with SMA should receive annual influenza (after 6 months of age) and pneumococcal vaccinations. In addition, for the first 24 months, infants diagnosed with SMA should receive therapy to prevent respiratory syncytial virus infection (palivizumab).
Delayed Diagnosis of SMA Type 3: A Case Study Clay, a 12-year-old budding athlete, presents to his pediatrician having met every developmental milestone up to this point but showing subtle changes in leg strength. His mother notices changes in his running stride. In addition, he has fallen, both on and off the field, and has difficulty pedaling a bike. At age 13 years, Clay notices a slight tremor in his hands during meals. The tremors are worse with purposeful activity. He is diagnosed with essential tremor by his pediatrician. At age 16 years, Clay presents with progressive weakness in his legs. He struggles to get up from a kneeling position on the lacrosse field. His pediatrician observes physical changes, but Clay blames his weakness on a lack of conditioning. That same year, obvious atrophy is apparent in his upper thighs and his pediatrician notes a difference in the muscular tone of his quadriceps versus his lower legs. At this point, 4 years after the initial visit to the primary care provider for the concern of a change in strength, Clay is referred to a pediatric neurologist. At the initial appointment, the pediatric neurologist reviews developmental milestones, and Clay’s mother explains the
history of falling, hand tremors, change in running stride, and inability to pedal a bike or get up from a kneeling position. Clay continues to deny that anything is wrong, saying he does not notice any physical changes. During the exam, the neurologist notes markedly impaired quadriceps strength and hypotonic reflexes in Clay’s lower extremities.The neurologist expresses concern that the patient may have a degenerative muscular disorder and orders laboratory tests, including creatine kinase, which is elevated to 4 times the normal level. The neurologist refers the patient to the Mayo Clinic for further evaluation by a neuromuscular specialist. After the second specialty consult, EMG is scheduled to rule out SMA. The EMG findings suggest a neurologic source of the muscular weakness, which narrows down the differential diagnosis.The final and definitive diagnosis for SMA is obtained from blood work showing a SMN1 gene mutation and 4 SMN2 copies. This case provides an example of diagnostic delays in a patient with SMA type 3 who met all infant and childhood milestones and developed progressive weakness in adolescence.
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SPINAL MUSCULAR ATROPHY
Orthopedic professionals involved in SMA care help manage associated scoliosis, which is more common in SMA types 1 and 2.1,2 Patients may have chest deformity and hip instability that require treatment by an orthopedic specialist.2 Patients with limited mobility are at higher risk for osteoporosis and vitamin D deficiencies related to disuse and may experience fragility fractures. The multidisciplinary team caring for patients with SMA includes speech therapists and nutritional specialists, who help to manage impaired swallowing and dysphagia.1,10 Regular growth and development assessments, including weight, height, and length measurements, are essential to help guide nutritional status. Additional gastrointestinal issues commonly include gastroesophageal reflux, constipation, and delayed gastric emptying, which may require surgical or pharmacologic interventions. Maintenance of a healthy weight is critical for patients with SMA because extra weight reduces mobility and increases the risk for comorbidities such as high blood pressure and diabetes and can exacerbate respiratory and orthopedic issues.1,2 Nonambulatory patients are at high risk for obesity because of a lack of physical activity.2 Spinal muscular atrophy is a complex disease requiring lifetime management involving a multidisciplinary team of health care providers. It also requires effective communication between the health care team, the patient, and their family as much of the care for patients with SMA types 1 and 2 is provided in the home. Although SMA types 3 and 4 may be less severe, patients and their health care providers need to be aware of how to manage this chronic disorder and optimize QOL. Knowing the essential assessments for monitoring disease progression, treatment outcomes, and potential complications is key to proactively caring for patients with SMA. ■
3. Butchbach ME. Copy number variations in the survival motor neuron genes: implications for spinal muscular atrophy and other neurodegenerative diseases. Front Mol Biosci. 2016;3:7. 4. Sugarman EA, Nagan N, Zhu H, et al. Pan-ethnic carrier screening and prenatal diagnosis for spinal muscular atrophy: clinical laboratory analysis of >72,400 specimens. Eur J Hum Genet. 2012;20(1):27-32. 5. Kraszewski JN, Kay DM, Stevens CF, et al. Pilot study of population-based newborn screening for spinal muscular atrophy in New York state. Genet Med. 2018;20(6):608-613. 6. Chien YH, Chiang SC, Weng WC, et al. Presymptomatic diagnosis of spinal muscular atrophy through newborn screening. J Pediatr. 2017;190: 124-129.e1. 7. Arnold WD, Kassar D, Kissel JT. Spinal muscular atrophy: diagnosis and management in a new therapeutic era. Muscle Nerve. 2015;51(2):157-167. 8. Centers for Disease Control and Prevention. Assessment: Timed Up & Go (TUG). Accessed June 30, 2021. https://www.cdc.gov/steadi/pdf/TUG_ Test-print.pdf 9. London Health Sciences Centre. Critical Care Trauma Centre. Assessment of motor function. Accessed June 30, 2021. https://www.lhsc.on.ca/criticalcare-trauma-centre/assessment-of-motor-function 10. Finkel RS, Mercuri E, Meyer OH, et al. Diagnosis and management of spinal muscular atrophy: part 2: pulmonary and acute care; medications, supplements and immunizations; other organ systems; and ethics. Neuromuscul Disord. 2018;28(3):197-207. 11. Spinraza (nusinersen) injection, for intrathecal use. Prescribing information. Biogen; 2020. Accessed June 30, 2021. https://www.spinraza.com/content/dam/ commercial/spinraza/caregiver/en_us/pdf/spinraza-prescribing-information.pdf 12. Zolgensma (onasemnogene abeparvovec-xioi) suspension, for intravenous infusion. Prescribing information. AveXis; 2021. Accessed June 30, 2021. https://www.novartis.us/sites/www.novartis.us/files/zolgensma.pdf 13. Evrysdi (risdiplam) for oral solution. Prescribing information. Genentech; 2020. Accessed June 30, 2021. https://www.gene.com/download/pdf/evrysdi_ prescribing.pdf
Mellisa Hall, DNP, AGPCNP-BC, FNP-BC, is a professor of nursing and chair of the Master of Science in Nursing Program at University of Southern Indiana College of Nursing and Health Professions, in Evansville, Indiana; Jennifer Titzer Evans, DNP, RN, NC-BC, is an associate professor of nursing and program chair of the undergraduate nursing program at the University of Southern Indiana. References 1. Mercuri E, Finkel RS, Muntoni F, et al. Diagnosis and management of spinal muscular atrophy: part 1: recommendations for diagnosis, rehabilitation, orthopedic and nutritional care. Neuromuscul Disord. 2018;28(2):103-115. 2. Prior TW, Leach ME, Finanger E. Spinal muscular atrophy. In: Adam MP, Ardinger HH, Pagon RA, et al, eds. GeneReviews. University of Washington;
More Ways to Find Us Like us on Facebook facebook.com/TheClinicalAdvisor Follow us on Twitter @ClinicalAdvisor Visit us on the web ClinicalAdvisor.com Download the app ClinicalAdvisor.com/App
1993-2021. Updated December 3, 2020. Accessed June 30, 2021. https://www.ncbi.nlm.nih.gov/books/NBK1352/
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FEATURE: CORINNE I. ALOIS, MS, PA-C; ALYSSA C. QUINLAN, PA-C, MPAS
Clinical Challenge: An Underdiagnosed Cause of Resistant Hypertension Once thought to be rare, the prevalence of primary aldosteronism is estimated to be 5% to 17%, although the condition remains underdiagnosed.
© AXEL KOCK / SCIENCE SOURCE
30-year-old woman presents to the emergency department (ED) with headache, nausea, vomiting, diarrhea, and abdominal pain that started 1 day ago. The patient reports 3 episodes of nonbilious, nonbloody vomiting with concomitant bilateral extremity weakness lasting about 15 minutes and 2 episodes of nonbloody diarrhea. She reports intermittent episodes of generalized body aches and lower extremity weakness over the last 2 months. She denies fevers, chills, weight loss, neck pain, shortness of breath, palpitations, chest pain, or paresthesias. The patient has a medical history of hypertension for 3 years and type 2 diabetes for 4 years. She has been noncompliant with medications for these conditions for the last 6 months because of insurance issues. It is not known whether she was screened for a secondary cause of hypertension when she initially began treatment. Her family history is noncontributory. She denies smoking as well as alcohol or drug use. She is a married stay-at-home mom with 2 daughters. She denies a history of gestational hypertension or preeclampsia but had a spontaneous abortion at 9 weeks’ gestation within the last year.The patient reports possible recent influenza exposure and is anxious that something is wrong. Up to 10% of patients with hypertension have aldosteronism.
On arrival, her vitals are as follows: blood pressure, 195/126 mm Hg; respiratory rate, 18 breaths per www.ClinicalAdvisor.com • THE CLINICAL ADVISOR • JULY/AUGUST 2021 35
CLINICAL CHALLENGE: PRIMARY ALDOSTERONISM
Primary aldosteronism is independent of the RAAS and is defined as excessive production of aldosterone by the adrenal glands. minute unlabored; heart rate, 71 beats per minute; temperature, 98.4 °F; and oxygen saturation, 97% on room air. On physical examination, the patient is somnolent but easily arousable. She is oriented, and her thoughts are coherent. She is of average build with a BMI of 24.8. Her skin is warm and her mucous membranes are moist. She has sweaty palms but no rashes or lesions. Her eyes appear normal with no proptosis. Visual acuity also is normal and extraocular movements are intact. A small, diffusely enlarged goiter (grade 1 according to World Health Organization classification) is found. A bruit is audible over the goiter but no tenderness or lymphadenopathy is apparent. Her lungs are clear to auscultation. Her heart examination reveals clear S1 and S2 without murmur, rubs, or gallops. Her abdomen is flat; no tenderness, masses, or organomegaly are present. Bowel sounds are normoactive. Pelvic and rectal examinations are normal. Peripheral pulses are intact. No edema is noted in the lower extremities. The patient has full range of motion, both active and passive, of the hands, wrists, elbows, shoulders, hips, knees, and ankles. Her strength is 5/5 in the upper and lower extremities bilaterally. Sensations of pain, light touch, proprioception, and vibration are intact. All reflexes are 2+. Laboratory Results
Serum laboratory values are remarkable for a low potassium level, high arterial pH, and borderline high bicarbonate level (Table). Anion gap, blood glucose, hemoglobin A1c, and lowdensity lipoprotein cholesterol levels are elevated. Complete blood cell count, troponin level, and coagulation studies are unremarkable. Serum pregnancy test is negative. The patient’s electrocardiogram reveals normal sinus rhythm at 71 beats per minute with left axis deviation and T-wave inversion in the inferior lateral leads. Lung fields are clear on chest radiograph. The patient is admitted to the cardiology service and immediately is started on labetalol 100 mg orally for elevated blood pressure. She shows little improvement and standing orders for hydralazine and lisinopril are written. Her blood pressure remains elevated; the hydralazine dosage is increased from 50 to 75 mg and, finally, to 100 mg 3 times a day with the goal of lowering her blood pressure to less than 130/80 mm Hg. She also receives acetaminophen for pain and ondansetron for nausea and/or vomiting as needed. The patient refuses
prophylactic therapy for influenza exposure but consents to influenza vaccination. An echocardiogram evaluation shows a left ventricular ejection fraction of 65% (normal range, 55%-70%); her valves and anatomy are of normal size and function. An endocrinologist is consulted for her uncontrolled diabetes and goiter.The patient is started on insulin lispro and insulin glargine.An ultrasound of the thyroid shows a 1.7-cm nodule on the left lobe and a 5.2-cm nodule on the right. However, laboratory evaluation of thyroid status is unremarkable. The patient’s hypokalemia is treated with 3 doses of potassium chloride powder (40 mEq orally every 4 hours). The patient’s plasma aldosterone level is 24.7 ng/dL (normal range, 1-21 ng/ dL) and plasma renin concentration is undetectable (normal range with a normal sodium diet, 0.6-4.3 ng/mL/h). Primary aldosteronism (PA) is identified as a potential cause of the patient’s illness and a contrast-enhanced computed tomography (CT) of the abdomen is ordered to evaluate for adrenal masses. The CT shows a left-sided adrenal mass measuring 2.5 cm × 1.8 cm. To confirm the diagnosis of PA, a saline infusion test is ordered. Consistent with PA, the plasma aldosterone TABLE. Abnormal Laboratory Results Measure
Anion gap, mEq/L
Hemoglobin A1c, %
LDL-C, low-density lipoprotein cholesterol a Optimal level
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PA is a leading cause of secondary hypertension, accounting for 5% to 10% of patients with hypertension and 20% of those with resistant cases. concentration increases to 38.7 ng/dL at the 4-hour mark. The patient’s symptoms improve and she is discharged on an angiotensin-converting enzyme (ACE) inhibitor for hypertension and a potassium supplement. She receives diabetes nutritional education during the hospitalization and insulin training. She is discharged on insulin injection and instructed to follow up as scheduled with the endocrine clinic. The patient is considering the recommendation to biopsy the thyroid nodules. A moderate-intensity statin, (atorvastatin 20 mg daily) is prescribed for hyperlipidemia. She is counseled to follow a low-fat, heart-healthy diet and avoid pregnancy while taking the statin. The patient has an intrauterine device for contraception.
lead to complaints of headaches and, if left untreated, may cause hypertensive retinal changes, stroke, renal insufficiency, hypertensive encephalopathy, and cardiac failure.6 Despite having an elevated blood pressure, patients with PA do not exhibit peripheral edema.4 Patients may be hypokalemic, even in the absence of potassium-depleting diuretics.4 Individuals who are severely hypokalemic may present with fatigue, muscle weakness, ileus, and palpitations.2 Severe loss of potassium may lead to hypokalemia-induced nephrogenic diabetes insipidus, which can cause polydipsia and/or polyuria.2 Other signs and symptoms include constipation, decreased deep tendon muscle reflexes, and hypomagnesemia.2 Diagnosis of Primary Aldosteronism
Once considered a rare condition, PA has emerged as a primary cause of secondary hypertension, accounting for 5% to 10% of patients with hypertension and 20% of those with resistant hypertension.1-4 The prevalence of PA is estimated to be 5% to 17%, although this condition is underdiagnosed.1,2 Hyperaldosteronism can be classified as primary or secondary. Secondary hyperaldosteronism is caused by excessive activation of the renin-angiotensin-aldosterone system (RAAS) caused by extra-adrenal stimuli, such as a renin-producing tumor, renal artery stenosis, or edematous disorders (eg, heart failure, pregnancy).2,4 Primary aldosteronism is independent of the RAAS and is defined as excessive production of aldosterone by the adrenal glands.2,4 Excessive production of aldosterone leads to increased activity of the sodium-potassium pumps in the cortical collecting ducts of the nephron, which, in turn, causes sodium retention and potassium loss.4 Hyperaldosteronism also causes increased loss of hydrogen ions in medullary collecting ducts, resulting in metabolic alkalosis.2 Causes of PA include: • Aldosterone-producing adrenal adenomas (Conn syndrome): these adenomas typically are unilateral but may be bilateral4 • Adrenal hyperplasia: this may be unilateral or bilateral; hyperplasia is more common among older men2,4 • Familial PA: this condition is rare and often related to germline mutations of electrolyte channels2,5 • Ectopic aldosterone-secreting tumors2 • Aldosterone-producing adrenocortical carcinoma2 The most common clinical manifestation of PA is hypertension that often is resistant to treatment.2,5 Hypertension may
The diagnostic approach to PA begins with routine laboratory studies to assess serum levels of sodium, potassium, magnesium, bicarbonate, glucose, blood urea nitrogen, and creatinine. Plasma aldosterone concentration (PAC) and plasma aldosterone to renin ratio (ARR) serve as screening tests for PA and play an important role in distinguishing between primary and secondary hyperaldosteronism.5 Normal levels of PAC are less than 10 ng/dL2; however, in PA, elevated levels of plasma aldosterone with decreased or normal renin activity increase the ratio. An ARR greater than 30 ng/dL and PAC greater than 20 ng/dL are highly suspicious for PA, with sensitivity and specificity of greater than 90%.2 Confirmatory diagnostic tests include a saline infusion test or oral sodium loading. • Saline infusion test: Patient is either sitting or lying down for at least 1 hour before and during a 4-hour infusion of IV normal saline. Plasma levels for renin, aldosterone, cortisol, and potassium are drawn at hours 0 and 4. Aldosterone levels of less than 10 ng/dL after the infusion can rule out the diagnosis of PA because levels do not decrease as they should in patients with PA. Cortisol levels are taken to exclude adrenocorticotropic hormone is not causing elevations in aldosterone levels.2 • Oral sodium loading test: Patients ingest 10 g to 12 g of sodium chloride orally for 3 days before testing. At the end of 3 days, serum and 24-hour urine are collected and tested for aldosterone, sodium, and potassium levels. Urine aldosterone levels greater than 14 µg/24 hours are confirmatory for PA. Urine sodium levels are tested to ensure adequate salt loading for proper interpretation of the test.2 To distinguish between causes of PA, thin-sliced adrenal CT with contrast is the most common initial diagnostic test to
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CLINICAL CHALLENGE: PRIMARY ALDOSTERONISM
For unilateral adrenal adenomas or hyperplasia, surgical resection is often curative. Bilateral adrenal hyperplasia requires a medical approach. identify aldosteronomas and adrenal cell carcinoma.5 Levels of aldosterone in adrenal venous blood may be measured in patients in whom there is high suspicion of unilateral PA despite normal imaging.⁵
References 1. Monticone S, Burrello J, Tizzani D, et al. Prevalence and clinical manifestations of primary aldosteronism encountered in primary care practice. J Am Coll Cardiol. 2017;69(14):1811-1820. 2. Dominguez A, Muppidi V, Gupta S. Hyperaldosteronism. In: StatPearls.
Treatment of Primary Aldosteronism
StatPearls Publishing; 2021. Updated November 21, 2020. Accessed June 22,
The overall treatment goal in PA is to reduce the morbidity and mortality associated with hypertension, hypokalemia, and other associated symptoms.5 The approach to treatment depends on the etiology of the PA. For unilateral adrenal adenomas or adrenal hyperplasia, surgical resection often is curative.5 Patients diagnosed with bilateral adrenal hyperplasia and familial PA require a medical approach. They should be started on an aldosterone antagonist such as spironolactone or eplerenone.5 For patients with persistent hypertension, clinicians should initiate another antihypertensive agent, such as hydrochlorothiazide or an ACE inhibitor. In addition, they should advise patients to follow a low-salt diet.6 Early diagnosis and treatment of PA can improve a patient’s overall long-term morbidity and mortality. Patients with PA have a higher risk of developing chronic kidney disease than individuals with essential hypertension. However, when surgical adrenalectomy is performed, there is no difference in kidney morbidity.7 Patients with PA also have an increased risk for stroke, atrial fibrillation, heart failure, coronary artery disease, diabetes, and metabolic syndrome than patients with essential hypertension.8
Primary aldosteronism should be considered in any patient with treatment-resistant hypertension, especially in a hypokalemic patient who is not taking a diuretic. Hypertension, unexplained hypokalemia, and metabolic alkalosis are the most common findings. The workup includes serum electrolytes (to evaluate for hypokalemia, metabolic alkalosis, hypernatremia, and hypomagnesemia), ARR screening, as well as a saline infusion or oral sodium loading test and adrenal CT with contrast. Individuals with PA have increased long-term cardiovascular and renal risks, making early diagnosis and treatment essential. ■
3. Brown JM, Siddiqui M, Calhoun DA, et al.The unrecognized prevalence of primary aldosteronism: a cross-sectional study. Ann Intern Med. 2020;173(1):10-20. 4. Grossman AB. Primary aldosteronism (Conn syndrome). In: Merck Manual Professional Version. Merck & Co, Inc; 2020. Updated September 2020. Accessed June 22, 2021. https://www.merckmanuals.com/professional/ endocrine-and-metabolic-disorders/adrenal-disorders/primary-aldosteronism 5. Funder JW, Carey RM, Mantero F, et al. The management of primary aldosteronism: case detection, diagnosis, and treatment: an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab. 2016;101(5):1889-1916. 6. Iqbal AM, Jamal SF. Essential hypertension. In: StatPearls. StatPearls Publishing; 2021. Updated July 10, 2020. Accessed June 22, 2021. https://www. ncbi.nlm.nih.gov/books/NBK539859/ 7. Hundemer GL, Curhan GC, Yozamp N, Wang M, Vaidya A. Renal outcomes in medically and surgically treated primary aldosteronism. Hypertension. 2018;72(3):658-666. 8. Monticone S, D’Ascenzo F, Moretti C, et al. Cardiovascular events and target organ damage in primary aldosteronism compared with essential hypertension: a systematic review and meta-analysis. Lancet Diabetes Endocrinol. 2018;6(1):41-50.
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Corinne I. Alois, MS, PA-C, is an assistant professor at St. John’s University Physician Assistant Program in Jamaica, New York; Alyssa C. Quinlan, PA-C, MPAS, is an assistant professor at St. John’s University Physician Assistant Program. 38 THE CLINICAL ADVISOR • JULY/AUGUST 2021 • www.ClinicalAdvisor.com
Dermatology Clinic CASE #1
Patches of Hair Loss TIFFANEY TRAN, BS;TARA BRAUN, MD; CHRISTOPHER RIZK, MD
A 15-year-old girl presents with patches of hair loss on her scalp that have been present for the last 6 months. She reports that her scalp is not itchy or painful and denies any recent illnesses, diet changes, or stressful life events. She does not have any other medical conditions and takes no medications. Her uncle had a similar condition that started when he was in his 20s, but no one else in the family has experienced hair loss.The patient says she has not tried any treatments for the condition. On examination, she has several well-demarcated patches of hair loss on her frontal and parietal scalp, with no perifollicular erythema or scale. What is your diagnosis? Turn to page 40
Pink Pedunculated Nodule on Palm DOMINIQUE JACOBS, BS; KATRINA HANSEN, DO
A 65-year-old man of Middle Eastern descent presents with a tender, weeping mass on his right palm. Since appearing 8 months ago, the lesion has enlarged into a 0.9-cm, pink pedunculated nodule with abundant serous crust.The patient has no significant history of trauma. His medical history is significant for atrial fibrillation, hypertension, coronary artery disease, congestive heart failure, and lung cancer, which was treated with right lobectomy. It is unclear what medications were used to treat his lung cancer. His current medications include metoprolol, lisinopril, allopurinol, aspirin, and digoxin. The patient also presents with additional pink, scaly plaques on his right ankle, consistent with nummular eczema. What is your diagnosis? Turn to page 41 www.ClinicalAdvisor.com • THE CLINICAL ADVISOR • JULY/AUGUST 2021 39
Dermatology Clinic CASE #1
Alopecia areata (AA) is a s udden-onset autoimmune condition characterized by discrete areas of nonscarring hair loss.1 The extent of alopecia can range from a few patches of hair loss to complete loss of all scalp hair (alopecia totalis) or all body hair (alopecia universalis).1 The earliest known records of AA are found in ancient Egyptian papyruses dating back to 1500 BC. In these texts, a phrase that translates to “bite alopecia” is thought to describe patchy AA.2 The term alopecia was first mentioned by Hippocrates in approximately 400 BC; later Celsus (25 BC-50 AD) first described “areas of alopecia” in his medical writings.2 In these documents, Celsus used the term alopekia to describe bald spots in the scalp and beard. AA also has been referred to as porrigo decalvans, tinea decalvans, and phytoalopecia.2 In the United States, the lifetime prevalence of AA is estimated to be 2.5%.3,4 Disease onset may occur at any age, including early childhood, and both sexes are affected equally.5 The majority of cases develop before 40 years of age.4 Alopecia areata is thought to be caused by the breakdown of immune privilege (IP) in the hair follicle (HF) and the subsequent development of autoimmunity against the HF.6 Immune privilege refers to the ability of certain organs to evade potential harm from an inflammatory immune response.6 Normally, HFs produce a milieu of local factors that maintain their IP, but in patients with AA, this process breaks down.1 The underlying cause of AA remains unknown; it is hypothesized that susceptible patients may have defects in the HF or dysregulation of their immune system.6 Risk factors for AA include genetic susceptibility, concurrent autoimmune disease, and environmental factors.7 Individuals with a family history of AA are more likely to develop AA, but onset and severity are difficult to predict.6,7 Genome-wide association studies have identified numerous genetic loci associated with increased AA risk, especially in the human leukocyte antigen region.1,7 Alopecia areata is associated with other autoimmune diseases, such as vitiligo, inflammatory bowel disease, psoriasis, thyroid disease, rheumatoid arthritis, and systemic lupus erythematosus.1 In patients with a genetic predisposition, possible environmental triggers include emotional stress, physical injury, viral infection, drugs, and vaccinations; however, in the majority of cases, the precipitating factor cannot be identified.1
In addition to its genetic variability, AA also demonstrates phenotypic variability. Alopecia areata classically manifests as well-circumscribed round, hairless patches without perifollicular scaling or erythema, but other distinctive patterns may be observed.1 For instance, ophiasis denotes a band-like pattern of hair loss along the occipital and temporal margins, and sisaipho denotes the opposite, that is, extensive hair loss sparing the margins.1 As AA may affect any hair-bearing site, hair loss may occur in the beard area and may involve the eyebrows and eyelashes.1 It may target pigmented hairs and spare white hairs, or it may induce diffuse hair loss without discrete patches.1 In some patients, the disease may progress to alopecia totalis or alopecia universalis. Nail changes, such as pitting or trachyonychia, are associated with severe manifestations of AA.1 In AA, exclamation point hairs, a specific finding defined by proximal narrowing of the hair shaft, may be appreciated on dermoscopy in addition to nonspecific yellow dots, black dots, and dystrophic hairs.1 Although AA usually is clinically diagnosed, a skin biopsy may be performed if the diagnosis is uncertain.
For patients with patchy disease, intralesional corticosteroids administered over multiple sessions are preferred. Histopathology will show characteristic peribulbar and intrabulbar lymphocytic infiltrate. The infiltrate also may contain eosinophils, CD1+ cells, and CD8+ cells. Other histologic findings may include pigment incontinence and an increased number of miniaturized hair follicles.1 Depending on examination findings, the clinician may consider other causes of nonscarring alopecia in the differential diagnosis, such as tinea capitis, androgenetic alopecia, telogen effluvium, and trichotillomania. In contrast to AA, tinea capitis presents with pruritus, erythema, and scaling of the scalp.1 In patients with androgenetic alopecia, diffuse hair loss occurs in a sex-specific pattern, and in patients with trichotillomania, patches of hair loss are irregular in shape and contain broken hairs.1 Telogen effluvium presents as diffuse hair shedding as opposed to the discrete patches of hair loss seen in AA.1 There is no cure for AA and treatment options have variable efficacy.8 Patients may recover spontaneously but most treated patients experience relapse.1 For patients with patchy disease, intralesional corticosteroids administered
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over multiple sessions are preferred; for those with limited disease, topical corticosteroids alone may be beneficial. However, for patients with extensive or rapidly progressive disease, systemic corticosteroids may need to be considered, bearing in mind potential side effects. Topical options for AA include minoxidil, anthralin, and immunotherapy (eg, diphenylcyclopropenone).1,8 Promising results have been reported with novel targeted approaches, such as Janus kinase inhibitors that block the downstream signaling pathway of interferon-γ.8 With any of these treatment approaches, patient education and counseling are necessary to manage patient expectations. Given its unpredictability and cosmetic consequences, AA can be a devastating diagnosis that negatively affects quality of life. Psychological comorbidities include depression and anxiety, and patients may require professional support as they cope with the disease.1 The patient in this case was diagnosed with AA clinically and she was immediately started on corticosteroid injections, after which she experienced some hair regrowth. However, several months later she started developing new patches of hair loss for which she is undergoing additional corticosteroid injections. Tiffaney Tran, BS, is a medical student at Baylor College of Medicine, in Houston,Texas;Tara L. Braun, MD, is a resident in the Department of Dermatology at Baylor College of Medicine; and Christopher Rizk, MD, is a dermatologist affiliated with Baylor College of Medicine. References 1. Pratt CH, King LE, Messenger AG, Christiano AM, Sundberg JP. Alopecia areata. Nat Rev Dis Primers. 2017;3:17011. 2. Broadley D, McElwee KJ. A “hair-raising” history of alopecia areata. Exp Dermatol. 2019;29(3):208-222. 3. Benigno M, Anastassopoulos KP, Mostaghimi A, et al. A large cross-sectional survey study of the prevalence of alopecia areata in the United States. Clin Cosmet Investig Dermatol. 2020;13:259-266. 4. Mirzoyev SA, Shrum AG, Davis MDP, Torgerson RR. Lifetime incidence risk of alopecia areata estimated at 2.1% by Rochester Epidemiology Project, 1990-2009. J Invest Dermatol. 2014;134(4):1141-1142. 5. Fricke ACV, Miteva M. Epidemiology and burden of alopecia areata: a systematic review. Clin Cosmet Investig Dermatol. 2015;8:397-403. 6. Rajabi F, Drake LA, Senna MM, Rezaei N. Alopecia areata: a review of disease pathogenesis. Br J Dermatol. 2018;179(5):1033-1048. 7. Simakou T, Butcher JP, Reid S, Henriquez FL. Alopecia areata: a multifactorial autoimmune condition. J Autoimmun. 2019:98:74-85. 8. Renert-Yuval Y, Guttman-Yassky E. The changing landscape of alopecia areata: the therapeutic paradigm. Adv Ther. 2017;34(7):1594-1609.
Lobular Capillary Hemangioma
Lobular capillary hemangioma (LCH), also known as pyogenic granuloma, is a benign vascular tumor that most commonly arises in tissues such as the skin and mucous membranes and occasionally is found subcutaneously, intravascularly, or in the gastrointestinal tract.1,2 It can develop spontaneously in sites of injury or within capillary malformations.1 Lobular capillary hemangioma typically begins as a small, red papule that may grow over weeks to months before stabilizing in size. The pedunculated papule is friable and often undergoes ulceration.3 A collarette of scales often is present at the base of the lesion, but this sign is not unique to this entity.3 The lesions can bleed profusely after unavoidable trauma.3 The diameter varies from a few millimeters to several centimeters, and satellite lesions may develop around a primary lesion or disseminate.3 Mucosal LCH is seen most commonly within the oral cavity.3 Cutaneous LCH typically appears on the trunk and extremities in adults and on the head and neck region in children.3 Histologic examination may be warranted to diagnose LCH. If the lesion is excised, it should be sent for histopathologic confirmation to rule out malignancy. Histologically, LCH consists of aggregates of capillary-sized vessels that develop within highly vascular granulation tissue with lobules of thin-walled capillaries embedded within a loose fibrous stroma.3 Scattered fibroblasts and inflammatory infiltrate often are found.3 The overlying epidermis is often thinned and develops erosion and ulceration in more severe cases.3 These lesions are neither pyogenic nor granulomatous; thus, the term pyogenic granuloma is not accurate. To correct the misleading terminology, Mills et al suggested using the more descriptive term lobular capillary hemangioma.4 Atypical lesions may necessitate the use of immunohistochemistry staining to rule out other causes.3 These lesions stain positive for vascular markers such as CD31, CD34, and factor VIII antigen but negative for glucose transporter-1, unlike infantile hemangioma.3,5 Pyogenic granuloma may occur in all age groups and there is no clear gender predominance.2 Reports conflict regarding the epidemiologic pattern of disease.2,6,7 The cause of LCH is unknown and no single pathway has been defined for pathogenesis of the lesions. Proposed mechanisms include an imbalance of proangiogenic and
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Dermatology Clinic antiangiogenic factors that leads to rapid proliferation of neovascular, friable, and lobular capillaries.3 Reactive granulation tissue from minor trauma and other predisposing factors, such as infection and preexisting vascular malformations, may contribute to pathogenesis.3 In pregnant patients, hormonal factors appear to play a role.2,8 Certain variants of LCH are associated with medication use. The most common medication-associated variant is multiple periungual pyogenic granuloma, which also is found in patients with other chronic dermatoses, such as atopic dermatitis and psoriasis.3 Drugs implicated most often include systemic and topical retinoids and antiretroviral, antineoplastic, and immunosuppressive agents.3 It is unclear whether the presenting patient had received chemotherapy or any other potentially causative agents previously because he did not follow up after biopsy and diagnosis.
Dominique Jacobs, BS, is a fourth-year medical student and aspiring dermatologist attending the Philadelphia College of Osteopathic Medicine; Katrina Hansen, DO, is the chief dermatology resident at Beaumont Farmington Hills in Michigan. Her interests include medical and surgical dermatology. References 1. Wollina U, Langner D, França K, Gianfaldoni S, Lotti T, Tchernev G. Pyogenic granuloma - a common benign vascular tumor with variable clinical presentation: new findings and treatment options. Open Access Maced J Med Sci. 2017;5(4):423-426. 2. Plachouri KM, Georgiou S. Therapeutic approaches to pyogenic granuloma: an updated review. Int J Dermatol. 2019;58(6):642-648. 3. Sarwal P, Lapumnuaypol K. Pyogenic granuloma. In: StatPearls. StatPearls Publishing; 2021. Updated December 5, 2020. Accessed June 28, 2021. https:// www.ncbi.nlm.nih.gov/books/NBK556077 4. Mills SE, Cooper PH, Fechner RE. Lobular capillary hemangioma: the
Lobular capillary hemangioma may necessitate treatment to alleviate discomfort, bleeding, or tissue obstruction.
underlying lesion of pyogenic granuloma. A study of 73 cases from the oral and nasal mucous membranes. Am J Surg Pathol. 1980;4(5):470-479. 5. Seyedmajidi M, Shafaee S, Hashemipour G, Bijani A, Ehsani H. Immunohistochemical evaluation of angiogenesis related markers in pyogenic granuloma of gingiva. Asian Pac J Cancer Prev. 2015;16(17):7513-7516. 6. Harris MN, Desai R, Chuang TY, Hood AF, Mirowski GW. Lobular capillary hemangiomas: an epidemiologic report, with emphasis on cutaneous lesions.
The differential diagnosis of LCH should include certain red flag lesions such as melanoma, squamous cell carcinoma, basal cell carcinoma, angiosarcoma, and malignant lymphoma. In an immunosuppressed individual, bacillary angiomatosis and Kaposi sarcoma should be included in the differential diagnosis. Benign lesions that present similarly to LCH include other capillary hemangiomas, irritated nevi, Spitz nevi, warts, granulation tissue from minor trauma, glomus tumor, and angiolymphoid hyperplasia with eosinophilia.1,3 Histopathology will help differentiate these conditions. Despite its benign nature, LCH may necessitate treatment to alleviate discomfort, bleeding, and/or obstruction of other structures. The selection of treatment should be made individually, depending on the size and severity of the lesions as well as patient characteristics and preferences. Treatment typically consists of excision, which results in the lowest rate of recurrence.1 Findings by Akamatsu et al suggest that shave excision followed by carbon dioxide laser ablation is linked to lower recurrence rates and is significantly less painful than excision and suture.9 Other treatments include curettage, electrocautery, radiosurgery, cryosurgery, sclerotherapy, and laser treatment. Successful photodynamic therapy with 5-aminolevulinic acid has been reported for a single LCH lesion on a finger; however, the advantage of this treatment compared with laser ablation has not been proven.1 ■
J Am Acad Dermatol. 2000;42(6):1012-1026. 7. Koo MG, Lee SH, Han SE. Pyogenic granuloma: a retrospective analysis of cases treated over a 10-year. Arch Craniofac Surg. 2017;18(1):16-20. 8. Jafarzadeh H, Sanatkhani M, Mohtasham N. Oral pyogenic granuloma: a review. J Oral Sci. 2006;48(4):167-175. 9. Akamatsu T, Hanai U, Kobayashi M, Miyasaka M. Pyogenic granuloma: a retrospective 10-year analysis of 82 cases. Tokai J Exp Clin Med. 2015;40(3):110-114.
42 THE CLINICAL ADVISOR • JULY/AUGUST 2021 • www.ClinicalAdvisor.com
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Dermatologic Look-Alikes Erythematous Plaques DINA ZAMIL, BS; TARA L. BRAUN, MD; CHRISTOPHER RIZK, MD
A 50-year-old man presents to the dermatology clinic with a 1-week history of an itchy rash. He has tried hydrocortisone cream on the rash, which has helped relieve the itching. His wife, who has been helping to apply the steroid cream, noticed that the lesions seem to come and go and have moved to different locations on the patient’s back. The patient takes medications for hypertension and hyperlipidemia but denies starting any new medications recently. On examination, there are several well-demarcated erythematous plaques with central pallor on the lower back, buttocks, and ankles, with nearby heme-crusted excoriations.
A 15-month-old boy with a 2-day history of worsening rash presents to the emergency department with his parents. He recently was diagnosed with otitis media by his primary care provider, for which he was given amoxicillin. His parents report that the rash began about a week after the boy started taking amoxicillin and has been progressively worsening. No prescription or over-the-counter treatments have been tried for the rash, and the patient has no other significant medical history. On examination, the patient has well-demarcated erythematous plaques with central areas of violaceous discoloration on the face, chest, back, and upper and lower extremities.
www.ClinicalAdvisor.com • THE CLINICAL ADVISOR • JULY/AUGUST 2021 43
Dermatologic Look-Alikes CASE #1
Acute urticaria is a common pruritic condition that presents with transient wheals and swelling. Acute urticaria typically lasts 6 weeks or less whereas chronic urticaria lasts longer than 6 weeks.1,2 Urticaria was recognized in the fourth century BC by Hippocrates, who noticed an association between the condition and nettles, or Urtica.3,4 The modern term urticaria was introduced by Frank in 1792.3 Urticaria is one of the most common dermatologic conditions seen in emergency departments (EDs), with a lifetime prevalence ranging from 12% to 20%.1,2,4 Although, acute urticaria occurs in only 7.6% to 16% of patients with urticaria, the condition is more common than chronic urticaria in infants and toddlers.1 Acute urticaria also is more prevalent among individuals with atopic diseases, such as atopic dermatitis.4 Acute urticaria affects individuals of all ages but the average age at presentation is in the early 20s.1 Reports indicate a female preponderance for acute urticaria, except in young children; in this population, the condition affects boys and girls equally.1 Urticaria arises from release of histamine and other proinflammatory mediators from mast cells and basophils in the dermis.2 This release often is mediated by immunoglobin E (IgE) but can occur without IgE or immunologic activation.2 Attacks can be caused by food, medications, and infections, but approximately 30% to 50% of acute urticarial attacks are considered idiopathic.1 Drugs implicated in urticaria include sulfonamide and beta-lactam antibiotics, opioids, acetylsalicylic acid, and nonsteroidal anti-inflammatory drugs (NSAIDs).5 When acute urticaria occurs with an infection, it can be difficult to determine whether the attack was precipitated by the infection or a medication used to treat it. Other etiologies for acute urticaria include ingestion of Anisakis simplex nematode, contact with latex, insect bites, systemic lupus erythematosus, and thyroid disease.1 More recently, reports have indicated that infection with SARS-CoV-2 can manifest with acute urticaria.6-8 In children, the most common etiology is viral infections (eg, rhinovirus and Epstein-Barr virus), as well as streptococcal, urinary tract, and parasitic infections.2 Urticarial wheals range in size from small (several millimeters) to much larger and tend to be well-circumscribed and pruritic. The wheals can be serpiginous, round, or polymorphic, and they may be pale or erythematous.2 Urticaria can affect the
skin anywhere on the body and may become generalized, involving 50% or more of the patient’s body surface area.1,2 Associated pruritus in patients with acute urticaria may impair sleep and/or daily functioning.2 Although urticaria usually is self-limiting, acute attacks accompanied by systemic symptoms, such as wheezing, dizziness, cough, flushing, and breathlessness, may indicate anaphylaxis.1,2 Acute urticaria also may co-occur with angioedema.1 Diagnosis of urticaria typically is made based on history and physical examination.5 It is important to gather information about the timing, frequency, size, and pattern of the wheals, as well as medication and supplement use, recent travel, infections, and other potential causes.2,5 Physical examination also should include vital signs as well as a test for dermographism. An examination of the ears, nose, throat, eyes, abdomen, lymph nodes, and musculoskeletal system can help reveal etiology.2 Individual wheals are transient, usually coming and going within 24 hours, and resolve without blisters or skin changes. The transient nature of the lesions distinguishes acute urticaria from many skin conditions. Conditions to consider in the differential diagnosis include erythema multiforme, arthropod bites, acute contact dermatitis, cellulitis, prodromal lesions of autoimmune bullous diseases, polymorphic eruption of pregnancy, progesterone-induced dermatosis, and systemic lupus erythematosus.1,2 Acute urticaria also commonly is confused with systemic mastocytosis, which features additional organ involvement, and urticarial vasculitis, which features painful lesions lasting more than 48 hours that result in discoloration or skin bruising.5 Finally, the presence of wheezing or stridor, gastrointestinal symptoms, and dizziness may indicate anaphylaxis. A complete review of systems can help identify systemic illnesses.2 Biopsy of a urticarial wheal should demonstrate dermal edema with mixed inflammatory perivascular infiltrate consisting of lymphocytes, eosinophils, and neutrophils.4 Additional tests to consider include complete blood count, which may support an infectious etiology; erythrocyte sedimentation rate; and C-reactive protein levels, which can indicate infection, inflammation, or NSAID-induced urticaria.1 Treatment is not necessary in mild cases of acute urticaria. If treatment is warranted, the mainstays are H1 antihistamines and topical (1%-2%) menthol aqueous cream to help relieve itching. Underlying causes of urticaria, including infections and aggravating drug, allergens, or other triggers, should be identified and addressed. Severe cases may warrant oral corticosteroids, especially if the acute urticaria presents with angioedema or systemic symptoms.1 The patient in this case was started on a daily H1 antihistamine, which resolved his lesions after several days, and the rash has not recurred.
44 THE CLINICAL ADVISOR • JULY/AUGUST 2021 • www.ClinicalAdvisor.com
Serum Sickness–Like Reaction
Serum sickness-like reaction (SSLR) is a syndrome named for its clinical resemblance to serum sickness (SS); it was described originally by von Pirquet and Schick in 1905.9-12 SS is associated with the injection of equine antitoxin, streptokinase, antilymphocyte globulin, and spider and snake antivenom; it is a type III hypersensitivity reaction resulting from deposition of circulating immune complexes that also features inflammation and complement activation.9,13 In contrast, SSLR lacks circulating immune complexes and hypocomplementemia.9 Although SSLR was common among patients treated with horse serum for diphtheria and tetanus in the early to mid-1800s, the condition now is less common because of modernized immunization techniques.11 Approximately 7% of the population will experience SSLR, with hospitalized patients having an increased risk (10%-20%).14 Among children, cefaclor use is a risk factor for SSLR, with estimates of cefaclor-associated SSLR ranging from 0.024% to 0.5%.13,14 A cefaclor metabolite is thought to bind to tissue proteins, resulting in an SSLR inflammatory response in genetically susceptible patients.13 A study of hospitalized children with SSLR in Turkey found an average
patient age of 101 months and a mean hospital stay of 5 days.15 SSLR generally occurs as a reaction to a drug and, in contrast to SS, is not associated with internal organ involvement, hypocomplementemia, circulating immune complexes, vasculitis, or renal involvement.10,13 Beta-lactam antibiotics, particularly cefaclor, as well as sulfonamide antibiotics and minocycline are major etiologic agents.9,13 Additional potentially causative classes include but are not limited to antineoplastic, psychiatric, anti-inflammatory (eg, NSAIDs and antirheumatic agents), antithymocyte globulin, antiepileptic, antiarrhythmic, and antihypertensive drugs.11,14 Recent reports have linked SSLR with bupropion, mirabegron, monoclonal antibodies (omalizumab and rituximab), elexacaftor/ tezacaftor/ivacaftor, drotaverine, and ixekizumab.13,16-20 Clinical symptoms of SSLR typically arise 1 to 3 weeks after initial administration of the causative medication.9 Skin eruptions are one of the most common manifestations of SSLR, appearing in 90% of cases.14 Cutaneous eruptions usually involve erythema and well-demarcated urticarial lesions, often with a lavender hue in the center. These urticarial plaques are sharply marginated and frequently coalesce.14,21 Patients also may present with acral erythema and/or edema as well as arthritis and/or arthralgia. Polyarticular joints, such as the knees, hips, wrists, and ankles, generally are painful and swollen, often leading to the inability to walk. Additional symptoms include fever and lymphadenopathy.9,13,21 Rashes of SSLR tend to be milder than those of SS.9,10 Diagnosis of SSLR is made via physical examination and history, particularly recent medication usage.14 Laboratory
TABLE. Acute Urticaria vs Serum Sickness-Like Reaction Acute Urticaria1–8
Serum Sickness-Like Reaction9-21
• Well-circumscribed, pruritic wheals • Can co-occur with angioedema
• Urticarial plaques, often with a central lavender hue • Arthralgia and/or arthritis in polyarticular joints
• Average presenting age in the early 20s • More common than chronic urticaria in children • Female preponderance, except in young children
• More prevalent among hospitalized patients • Increased prevalence in children
Potential risk factors
• Atopic diseases
• Cefaclor use in children
• Immunoglobulin E-mediated mast cell histamine release • Food, medication, or infections (viruses, bacteria, and parasites) • Rarely caused by systemic conditions
• Drug reaction • Most common etiology is beta-lactam and sulfonamide antibiotics (cefaclor in children)
• Dermal edema with mixed inflammatory perivascular infiltrate
• Superficial, mid-dermal, interstitial, and perivascular infiltrate consisting of eosinophils and neutrophils
• Clinical via history and physical examination
• Clinical via history and physical examination
• Topical 1%-2% menthol aqueous cream • H1 antihistamines • Treat underlying cause • Oral corticosteroids in severe cases
• Self-limiting • Causative drug should be withdrawn and avoided • Severe cases may warrant hospitalization, oral corticosteroids
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Dermatologic Look-Alikes results can show mild hematuria or proteinuria, eosinophilia, leukocytosis, and elevated erythrocyte sedimentation rate.13,14 In cases where biopsy is obtained, histopathology features include superficial, mid-dermal, interstitial, and perivascular infiltrates consisting of eosinophils and neutrophils.14 Differential diagnosis for SSLR includes hypersensitivity vasculitis, drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome, toxic epidermal necrolysis, and StevensJohnson syndrome. Hypersensitivity vasculitis, which often is triggered by beta-lactam administration, causes cutaneous eruptions and joint involvement, but it appears clinically more similar to leukocytoclastic vasculitis, presenting with erythematous to violaceous papules on the lower extremities, potentially with blisters and small ulcers. DRESS syndrome results in facial edema and exanthema leading to secondary desquamation and does not involve joints. Toxic epidermal necrolysis and early Stevens-Johnson syndrome can be differentiated from SSLR by their mucosal involvement and rapid desquamation.14
References 1. Sabroe RA. Acute urticaria. Immunol Allergy Clin North Am. 2014;34(1):11-21. 2. Schaefer P. Acute and chronic urticaria: evaluation and treatment. Am Fam Physician. 2017;95(11):717-724. 3. Czarnetzki B. The history of urticaria. Int J Dermatol. 1989;28(1):52-57. 4. Zuberbier T. Urticaria. Allergy. 2003;58(12):1224-1234. 5. Kanani A, Betschel SD, Warrington R. Urticaria and angioedema. Allergy Asthma Clin Immunol. 2018;14(suppl 2):59. 6. Falkenhain-López D, Sánchez-Velázquez A, López-Valle A, Ortiz-Frutos FJ. SARS-coronavirus-2 and acute urticaria. Int J Dermatol. 2020;59(7):867-868. 7. Adeliño R, Andrés-Cordón JF, Aracelis De La Cruz Martínez C. Acute urticaria with angioedema in the setting of coronavirus disease 2019. J Allergy Clin Immunol Pract. 2020;8(7):2386-2387. 8. Abuelgasim E, Dona ACM, Sondh RS, Harky A. Management of urticaria in COVID-19 patients: a systematic review. Dermatol Ther. 2021;34(1):e14328. 9. Katta R, Anusuri V. Serum sickness-like reaction to cefuroxime: a case report and review of the literature. J Drugs Dermatol. 2007;6(7):747-748. 10. Peter JG, Lehloenya R, Dlamini S, et al. Severe delayed cutaneous and
Cefaclor use is a risk factor for SSLR, with estimates of cefaclor-associated SSLR ranging from 0.024% to 0.5%.
systemic reactions to drugs: a global perspective on the science and art of current practice. J Allergy Clin Immunol Pract. 2017;5(3):547-563. 11. Wolf R, Orion E, Marcos B, Matz H. Life-threatening acute adverse cutaneous drug reactions. Clin Dermatol. 2005;23(2):171-181. 12. von Cesanatico P, Freiherr CP, Schick B. Die Serumkrankheit. Franz Deuticke; 1905. 13. Knowles SR, Shear NH. Recognition and management of severe
SSLR characteristically is benign and self-limited and can be treated symptomatically, with treatment depending on the severity of the case.9-11 The offending drug should be discontinued and avoided in the future. Symptoms can be treated with topical corticosteroids, NSAIDs, and antihistamines.13,14 Patients with severe joint involvement or extensive rash may require oral corticosteroid therapy or hospitalization.9,13,21 After withdrawal of the aggravating drug, SSLR generally resolves within 1 to 2 weeks.14 In cases of cefaclor-induced SSLR, some clinicians avoid subsequent use of beta-lactam antibiotics, but administration of a different cephalosporin typically is well-tolerated.13 Patients who have had SSLR induced by minocycline should consider avoiding other tetracyclines because little is known about cross-reactivity.13 The patient in this case was treated in the ED with antihistamines and topical steroids. He showed significant improvement in his rash overnight and then resolution over the next week. He has experienced neither sequelae from the rash nor recurrence. ■
cutaneous drug reactions. Dermatol Clin. 2007;25(2):245-253, viii. 14. McNamara K, Hughes OB, Strowd LC. Cutaneous drug eruptions including serum sickness-like reaction, symmetrical drug-related intertriginous and flexural exanthema, and drug-induced lupus. Clin Dermatol. 2020;38(6):641-647. 15. Yorulmaz A, Akın F, Sert A, Ağır MA, Yılmaz R, Arslan Ş. Demographic and clinical characteristics of patients with serum sickness-like reaction. Clin Rheumatol. 2018;37(5):1389-1394. 16. Tan MG, Burns BF, Glassman SJ. Serum sickness-like reaction associated with mirabegron. JAAD Case Rep. 2019;5(6):537-539. 17. Weiss SL, Smith DM. A case of serum sickness-like reaction in an adult treated with omalizumab. Mil Med. 2020;185(5-6):e912-e913. 18. Brennan S, Marmor I, Schafer C, et al. Serum sickness-like reaction following initiation of elexacaftor/tezacaftor/ivacaftor therapy. Pediatr Pulmonol. 2020;55(11):2846-2847. 19. Ali S, Corcea SL, Cristian RM, Bumbacea RS. A rapid desensitization protocol in a case of drotaverine-induced serum sickness-like reaction in a pregnant woman: a case report. Exp Ther Med. 2019;18(6):5105-5107. 20. Lindberg MR, Todd SP, Bunker DR, DeKlotz CMC. Ixekizumab-induced
Dina Zamil, BS, is a medical student at Baylor College of Medicine, in Houston,Texas;Tara L. Braun, MD, is a resident in the Department of Dermatology at Baylor College of Medicine; and Christopher Rizk, MD, is a dermatologist affiliated with Baylor College of Medicine.
serum sickness (like reaction): an unusual adverse effect. J Clin Rheumatol. Published online December 3, 2019. 21. Bender NR, Chiu YE. Dermatologic evaluation of the patient. In: Nelson Textbook of Pediatrics. 21st ed. Elsevier. 2019:3441-3451.
46 THE CLINICAL ADVISOR • JULY/AUGUST 2021 • www.ClinicalAdvisor.com
LEGAL ADVISOR CASE
© MANGTENG / GETTY IMAGES
Prisoner’s Visit to ED Cues Suit Inmate with hand bite was discharged without antibiotics or radiograph.
ANN W. LATNER, JD
Mr S is an inmate at a local prison. During an altercation with another prisoner, Mr S was bitten on his left hand. The bureau of prisons sent him to a local emergency department (ED), where he was seen by Mr N, a nurse practitioner, and Dr D, the supervising physician. The patient was described as agitated and complained about pain from the bite to his left pinkie. Mr N was instructed by Dr D to give the patient a tetanus vaccine and discharge him with a 3-day supply of antibiotics. As Mr N was cleaning and sterilizing the wound and bandaging the finger, the patient noted that he thought his finger was broken and requested an “x-ray.” Dr D, who was walking by, told Mr S that the hospital’s imaging machine was broken and a radiograph of the finger could not be performed. Mr S’s wound was cleaned and dressed but not sutured to avoid infection. Despite Dr D’s orders, no antibiotics were noted on the ED discharge report, and the patient was returned to prison without the prescription. In prison, Mr S continued to complain about pain in his finger, which he described as 10 on a
Patient claims hospital staff discharged him without properly screening and stabilizing him, violating EMTALA.
pain scale of 1 to 10. Six weeks after his visit to the ED, Mr S’s finger was radiographed, which revealed a displaced dorsal rotated fracture through the distal shaft of the left fifth middle phalanx. The radiologist’s report also noted that “there is surrounding soft tissue swelling without joint space malalignment.” Mr S was taken to a hand specialist who noted that he had a “fracture through the middle phalanx with displacement and angulation.” The specialist recommended surgery; specifically,“left small finger P2 fixation with DIP arthrodesis.” Mr S underwent the surgery, but he remained angry about how he was treated initially by the hospital and staff. Dr D and Mr N were unaware of Mr S’s condition until they were notified by the hospital’s attorney that the hospital was being sued by Mr S, pro se (representing himself without an attorney). The basis of the claim was violation of the federal Emergency Medical Treatment and Active Labor Cases presented are based on actual occurrences. Names of participants and details have been changed. Cases are informational only; no specific legal advice is intended. Persons pictured are not the actual individuals mentioned in the article.
www.ClinicalAdvisor.com • THE CLINICAL ADVISOR • JULY/AUGUST 2021 47
LEGAL ADVISOR Act, commonly referred to as EMTALA. Mr S claimed that the hospital and staff had discharged him without properly screening and stabilizing him. The Court Decides
The hospital’s attorney filed a motion to dismiss, arguing that EMTALA was aimed at curbing patient dumping, and that wasn’t the case here. The court noted that “Although Congress was concerned that the indigent and uninsured tended to be the primary victims of patient dumping, EMTALA is not limited to these individuals. Rather, under EMTALA, any individual who suffers personal harm as a direct result of a hospital’s violation of the statute may bring a private civil action for damages.”
EMTALA requires screening and treatment to individuals seeking emergency care in a nondiscriminatory manner. The court pointed out that EMTALA requires hospitals to provide medical screening and stabilizing treatment to individuals seeking emergency care in a nondiscriminatory manner. “A hospital has 2 primary obligations under EMTALA: 1) if an individual arrives at an emergency room, the hospital must provide appropriate medical screening to determine whether an emergency medical condition exists; and 2) if the hospital determines an individual has an emergency medical condition that has not been stabilized, it may not transfer the patient unless certain conditions are met.” Because Mr S had alleged both failure to screen and failure to stabilize claims, the court looked at each separately. Regarding failure to screen, the court pointed out that EMTALA doesn’t define what constitutes an appropriate
medical screening, and so the screening provision generally has been interpreted as requiring hospitals to screen all similar patients according to the same procedures. It is up to the hospital what the screening procedures will be, but they must be applied to all patients. Mr S, who researched his case in the prison’s law library, found a personal injury case in which the person was treated at the same hospital for a remarkably similar injury. In that case, the patient was given a radiograph and the patient was sent to surgery, during which an orthopedic surgeon inserted a plate and 6 screws to repair the fracture. The hospital’s attorney tried to argue that a single case was not enough to prove that the patient was treated differently than other patients. However, the court held that at this early stage in the case, this evidence was sufficient to move forward. The court then looked at the claim of failure to stabilize. To establish this claim, the plaintiff must allege that he had an emergency medical condition, that the hospital actually knew of the condition, and that the patient was not stabilized before being transferred. The hospital’s attorney tried to argue that Mr S did not have an emergency medical condition as defined by EMTALA. However, the court pointed out that it could not say as a matter of law that Mr S did not have an emergency medical condition, and this would be something for a jury to decide. The court ultimately concluded that Mr S had, in fact, presented a failure to stabilize claim and that the case could proceed. The case is moving its way through the legal process. Protecting Yourself
Regardless of the social standing of a patient, all patients must be screened and stabilized the same way. ■ Ann W. Latner, JD, a former criminal defense attorney, is a freelance medical writer in Port Washington, New York.
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