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Pemphigus Foliaceous (PF)

  • Rare form of pemphigus, similar demographics to PV *except* Fogo Selvagem
  • Fogo Selvagem is endemic in South America (e.g. Brazil), North Africa, and Turkey with prevalence up to 3%
  • Linked to black flies (Simulium spp.)
  • Favors young adults and children in rural areas

  • Acantholysis in superficial epidermis; granular layer due to Dsg1 immunoglobulin G → Cutaneous only
  • No mucosal involvement (no Dsg3 antibody)
  • Neonates of mothers with PF do NOT develop blistering (neonatal skinlike mucosa, Dsg3)

NB: Staphylococcus Aureus exfoliative toxins cleave Dsg1 → superficial blister “bullous impetigo or staphylococcus scalded skin” (histologically similar to pemphigus foliaceus)

NB: Other diseases transmitted by black flies are onchocerciasis, filariasis

  • Superficial, small, scattered painful/burning blisters (rapidly erupt → crusts/erosions mainly seen) in seborrheic distribution (head, neck, trunk)
  • Nikolsky often +
  • May coalesce, may progress to erythroderma
Two variants
  1. Pemphigus erythematosus “Senear-Usher syndrome” affecting malar face → overlaps w/ cutaneous lupus
  2. Fogo selvagem or endemic pemphigus
    • Clinically, histologically, and immunopathologically = PF

H & E (lesional)
  • Early: eosinophilic spongiosis
  • Established: Acantholysis in granular layer +/- neutrophils in the cavity; mixed dermal infiltrate with eosinophils and neutrophils
DIF (perilesional)
  • Most sensitive, ~100% of patients
  • Intercellular IgG, cannot reliably distinguish from PV on DIF
Demonstration of circulating auto-antibodies by:
  • IIF *substrate is human skin OR guinea pig esophagus
  • Immunoblotting
  • Immunoprecipitation
  • ELISA (useful to monitor disease activity, more sensitive, and specific than IIF)
  • CLEIA

  • Similar to pemphigus vulgaris when active and widespread
  • Localized: Superpotent topical corticosteroids
  • Dapsone when neutrophils are dominant histologically

  • Impetigo
  • Other types of pemphigus
  • Subcorneal pustular dermatosis
  • Subacute cutaneous lupus erythematosus
  • Seborrheic dermatitis
  • Other photosensitive disorders if SenearUsher
  • Other causes of erythroderma if erythrodermic