Orphanet: Superficial pemphigus

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Superficial pemphigus

Disease definition

A rare, autoimmune, bullous skin disease characterized clinically by multiple flaccid blisters, typically occurring on the face, scalp, trunk and extremities, which rapidly evolve into scaly, crusted, pruritic skin erosions. Histopathologically, superficial acantolytic blisters, as well as intercellular deposits of IgG autoantibodies directed against desmoglein 1 (and occasionally against desmoglein 3) in the upper epidermis, are observed.


Classification level: Group of disorders
  • Synonym(s): -
  • Prevalence: 1-9 / 100 000
  • Inheritance: Not applicable 
  • Age of onset: All ages
  • ICD-10: L10.2  L10.3  L10.4
  • OMIM: -
  • UMLS: -
  • MeSH: -
  • GARD: -
  • MedDRA: -

Detailed information


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