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Essen%al Dermatopathology  

Immunopathology

h2p://www.interpathology.org

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Essen%al Dermatopathology  

h2p://www.interpathology.org

Immunodermatological Diseases Epidermis

Immunopathology

BM zone

IgG

IgA

Linear/ Granular

Linear/ Granular

Pemphigus

Intraepidermal neutrophilic IgA dermatosis

IgG Linear

Epidermal side

Dermal side

Pemphigoid/ Herpes gestationis

Epidermolysis bullosa acquisita

Dermis IgA

IgA

Granular

Linear

Granular

Lupus erythematosus

Linear IgA bullous dermatosis

Papillary dermis

Vessel walls

Dermatitis herpetiformis

Henoch-Schonlein purpura

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Essen%al Dermatopathology  

Immunopathology

h2p://www.interpathology.org

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Essen%al Dermatopathology  

Immunopathology

h2p://www.interpathology.org

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Essen%al Dermatopathology  

h2p://www.interpathology.org

Predominantly intraepithelial  

Intercellular (cell   surface)  

IgG

+ BMZ  

Granular IgG   and/or  other  Ig  

Pemphigus erythematosus*  

Immunopathology

IgA

-­‐ BMZ  

Linear C3  and/or   IgG  

Diffuse granular  

AnB-­‐Ro Ab   present  

IgA pemphigus   (intraepidermal   IgA  dermatosis)  

Pemphigus (all   forms)  

ParaneoplasBc pemphigus*  

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Essen%al Dermatopathology  

Immunopathology

h2p://www.interpathology.org

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Essen%al Dermatopathology  

h2p://www.interpathology.org

Predominantly BMZ  

Thick glassy  linear   (IgG,  oTen  IgA)  

Finely linear  

C3 only  

IgG (±  C3)  

Pemphigoid (all   forms),  HG  

Linear IgA  dermatosis   (including  chronic   bullous  disease  of   childhood)  

Salt split  

IgG on  “roof”  

Pemphigoid (all   forms),  HG  

Immunopathology

IgA

Porphyria cutanea   tarda  /   pseudoporphyria  

Granular

IgA

DermaBBs herpeBformis  

≥2 Ig  (lupus  band)  

? Lupus   erythematosus  

IgG on  “floor”  

EBA, bullous  LE,  rare   pemphigoid  

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Essen%al Dermatopathology  

Immunopathology

h2p://www.interpathology.org

8


Essen%al Dermatopathology  

Immunopathology

h2p://www.interpathology.org

9


Essen%al Dermatopathology  

Immunopathology

h2p://www.interpathology.org

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Essen%al Dermatopathology  

h2p://www.interpathology.org

Roof only  

Roof and  base  

Base only  

Bullous pemphigoid  (80%)   Bullous  pemphigoid  (15%)   Cicatricial  pemphigoid  

Cicatricial pemphigoid  

Cicatricial pemphigoid  

EB acquisita   Linear  IgA  dermatosis  

Linear IgA  dermatosis  

Linear IgA  dermatosis  

Bullous SLE   Porphyria  cutanea  tarda  

Immunopathology

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Essen%al Dermatopathology  

Immunopathology

h2p://www.interpathology.org

12


Essen%al Dermatopathology  

Immunopathology

h2p://www.interpathology.org

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Essen%al Dermatopathology  

h2p://www.interpathology.org

Predominantly vascular/ perivascular   Smooth,  thick   IgG  (±IgA)  +   BMZ  deposits   Porphyria   cutanea  tarda  /   pseudoporhyria  

Immunopathology

Granular IgA  

Henoch-­‐ Schönlein purpura  

Granular IgG  

VasculiBs

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Essen%al Dermatopathology  

h2p://www.interpathology.org

Granular IgA  around  superficial  venules:   Henoch-­‐Schönlein  purpura    

Immunopathology

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Essen%al Dermatopathology  

h2p://www.interpathology.org

Fibrinogen: Marker  for  vascular  permeability  only  

Immunopathology

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Immunopathology  

Teaching program on Essential Dermatopathology - Immunopathology

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