Pemphigus and Pemphigoid

Pemphigus vulgarisMucous membrane pemphigoid
Target proteins
– Autoimmune reaction
– Intercellular keratinocyte protein (desmoglein 3) destroyed by auto antibodies
– Autoimmune reaction to basement membrane proteins:
– Lamina 5
– BP 180
ClinicalIntraepithelial blistersSubepithelial blisters
Site– Oral mucosa
– Skin
– Mucosa
– Oral mucosa
– Conjunctiva
– Skin rarely
Histology– Intraepithelial clefting
– Loss of desmosomal contacts therefore free floating acantholytic Tzanck cells
– Subepithelial clefting
– Chronic inflammatory infiltrate
Direct immunofluorescence testingCirculating auto IgG No circulating auto IgG
Difference between pemphigus and pemphigoid

Pemphigus vulgaris

  • Autoimmune blistering disorder
  • Results in loss of integrity of intercellular attachments within epidermis and mucosal epithelium


  • P. Vulgaris (common)
  • P. Vegetans (wart like)
  • P. Foliaceus (flower like)
  • P. Erythematosus (reddened)

Nikolsky’s sign: Positive – stroke mucosa gently, vesicle or bulla appears

Management: Corticosteroids + Azathioprine + Cyclophosphamide

Prognosis: Good. Fatal if untreated

Immunofluorescence of pemphigus vulgaris

Mucous membrane pemphigoid

  • Autoimmune blistering disorder
  • Results in loss of integrity of dermal-epidermal junctions
  • Affects oral tissues in 30% of cases
  • More severe

Management: Corticosteroids

Prognosis: Fair

Differential diagnosis:

  • Erythematous lichen planus
  • Linear IgA disease
  • Discoid lupus erythematous
  • Contact allergy
immunofluorescence of mucous membrane pemphigoid

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