January/February 2018
Volume 16 • Issue 1
CASE STUDY
Pemphigus Vegetans Confined to the Face and Scalp Anissa Zaouak, MD;1 Houda Hammami, MD;1 Mariem Belhaj Salah, MD;2 Achraf Debbiche, MD;2 Samy Fenniche, MD1
A 58-year-old man presented to our department with multiple chronic vegetant plaques on the face and scalp that had been present for 4 months. The plaques were well-defined hypertrophic areas, measuring between 3 cm × 3 cm and 3 cm × 4 cm, that were located mainly on the face symmetrically and on the forehead (Figure 1). The patient did not report itching or pain. There were also dry, crusty, well-defined plaques on the scalp. No other lesions were present on the skin or mucous membranes. (SKINmed. 2018;16:60–61)
A
cutaneous biopsy showed marked epidermal papillomatosis, acanthosis, and hyperkeratosis with diffuse suprabasal cleavage. The dermis showed heavy infiltratation by lymphocytes and eosinophils (Figure 2). Direct immunofluorescence demonstrated epidermic intercellular deposits of immunoglobulin G. Antibodies against desmoglein 3 were also present. Clinical, histologic and immunopathologic findings allowed us to make a diagnosis of pemphigus vegetans confined to the face and scalp. We initiated prednisone 1.25 mg/kg per day with azathioprine 50 mg per day. After 4 months, there was complete resolution of the vegetating plaques. At the time of writing, the patient was still receiving maintenance therapy. DISCUSSION Pemphigus vegetans is a rare variant of pemphigus vulgaris, characterized by chronic vegetating erosions primarily in the flexures. It was first described by Isador Neumann (1832–1906) in 1876.1,2 It usually presents as heaped-up, cauliflower-like vegetating plaques in the flexures. Although most lesions are typically located in intertriginous spaces, any area of the integument can be affected. Involvement of the scalp, soles, genitalia, and nails has been described.3
Figure 1. Bilateral verrucous and scaly plaques on the cheek and forehead.
From the Department of Dermatology1 and the Department of Anatomopathology,2 Habib Thameur Hospital, Tunis, Tunisia Address for Correspondence: Anissa Zaouak, MD, 8 Street Ali Ben Ayed, Montfleury 1008, Tunis, Tunisia • E-mail: anissa_zaouak@yahoo.fr
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