• Type: Autoimmune blistering disorder
• Autoantibodies: Against Desmoglein-3 (mucosa) ± Desmoglein-1 (skin) — components of desmosomes
• Hypersensitivity type: Type 3 Hypersensitivity (Ag-Ab complex)
• Blister type- Flaccid, Intraepidermal blisters
Pemphigus group of disorders | Pemphigoid group of disorders | |
Bullae | Flaccid bullae | Tense bullae (because whole epidermis is present in bulla covering so bulla is tight / tense) |
Bullae Location | Intra epidermal | Sub epidermal |
Age of onset | 40-60 year | 60-80 year (old age) |
• Row of tombstone • Acanthocytes • Nikolsky sign • Mucosal involvement • Intra epidermal bullae | Present | Absent |
Bulla spread sign | Present | Present |
Mucosal involvement:
• Always present, commonly starts in the oral cavity
Skin Lesions:
• Painful, flaccid bullae that rupture easily → erosions
Nikolsky sign:
• Positive (gentle pressure causes skin to shear off)
Acantholysis:
• Seen on histology (loss of cohesion between keratinocytes)
Histology:
• Row of tombstones appearance (It refers to the appearance of basal keratinocytes that remain attached to the basement membrane after the upper layers of the epidermis split due to acantholysis)
Immunofluorescence:
• Intercellular IgG deposition in a “net-like” or “fishnet” pattern
Treatment:
• High-dose corticosteroids, immunosuppressants (azathioprine, rituximab)
Pemphigus Vulgaris | Pemphigus Foliaceus | |
DSG 3 > DSG 1 | Antigen | DSG1 |
Suprabasal | Split | Subcorneal |
Always present | Mucous memb involvement | Absent |
Crusted erosions Vesicle & Bullae | On skin | Crusted erosions (Seborrheic distribution) |
