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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  

4  


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  

6  


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  

10  


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  

13  


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