1.2.5 Pemphigus Vulgaris

Grading & Level of Importance: C






Incidence of 2-7 new cases per million per year. F = M, 30-60 years. Ethnic variations. 


Autoimmune disease with intraepidermal blister formation.

Aetiology & Pathogenesis

Loss of cell adhesion within epidermis (Acantholysis) and blister formation by circulating autoantibodies against desmosomal proteins (desmogleins 1 and 3).

Signs & Symptoms

Pemphigus vulgaris: onset with oral erosions in more than 50% of cases, later superficial, fragile blisters with rapid transition to crusted erosions. The lesions usually develop in non-inflamed skin. Nikolski's signs positive (Nikolski I: blisters induced by rubbing normal skin. Nikolski II: existing blisters extend with lateral pressure). Pemphigus foliaceus: It manifests with impetigo-like crusted erosions usually on an erythematous base, and usually starts in seborrheic areas. Mucosae are usually spared.


Oral mucosae and entire skin surface, especially head, neck and trunk.


  • Pemphigus vulgaris: target antigens desmoglein 3 and desmoglein 1, suprabasal blister formation. Rare variants include neonatal pemphigus, pemphigus vegetans.
  • Pemphigus foliaceus: target antigen desmoglein 1, subcorneal blister formation.
    • Fogo selvagem (Brazilian pemphigus): endemic form of pemphigus foliaceus in Brazil, black flies (Simulium) may be vectors.
    • Pemphigus erythematosus: pemphigus foliaceus with positive ANA and photosensitivity.
  • Paraneoplastic pemphigus: autoantibodies against both intraepidermal and subepidermal attachment proteins. Intraepidermal and subepidermal blister formation.
  • IgA pemphigus.
  • Pemphigus herpetiformis.

Laboratory & other workups

Direct immunofluorescence (DIF): identification of in vivo bound intraepidermal, intercellular autoantibodies in the skin biopsy. Indirect immunofluorescence (IIF): identification in patient's serum of circulating autoantibodies against stratified squamous epithelium. ELISA for detection of anti-desmoglein 1 and 3 an autoantibodies in serum.


Intraepidermal clefts secondary to acantholysis.


Chronic, progressive. If untreated may be fatal.


Before the introduction of systemic corticosteroids, high mortality rate (skin-related sepsis, marasmus secondary to fluid and protein loss, difficulty in eating). Today complications secondary to high-dose corticosteroids and other immunosuppressive therapy.


Tzanck test: direct identification of acantholytic cells in smear of blister content (smear, Giemsa stain). Histology (acantholysis). DIF: intraepidermal intercellular IgG deposits. IIF: circulating IgG, which binds to stratified squamous epithelium in intercellular pattern. Immunoblot/ELISA: antibodies anti-desmoglein 1 and 3 autoantibodies in serum.

Differential diagnosis

Prevention & Therapy

Systemic: rituximab, long-term immunosuppressive therapy (corticosteroids, azathioprine, methotrexate, dapsone, cyclophosphamide). In refractory cases: High dose intravenous immunoglobulins. Topical: general wound care.

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