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Dystrophic epidermolysis bullosa

Disease definition

A group of inherited epidermolysis bullosa (EB) characterized by cutaneous and mucosal fragility resulting in blisters and superficial ulcerations that develop below the lamina densa of the cutaneous basement membrane and that heal with significant scarring and milia formation. Dystrophic epidermolysis bullosa (DEB) comprises four major and several rare sub-types with the three most common being intermediate dominant DEB, severe recessive DEB and intermediate recessive DEB.


Classification level: Group of disorders
  • Synonym(s):
    • DEB
    • Dermolytic epidermolysis bullosa
    • Epidermolysis bullosa dystrophica
  • Prevalence: 1-9 / 1 000 000
  • Inheritance: Autosomal dominant or Autosomal recessive 
  • Age of onset: Infancy, Neonatal
  • ICD-10: Q81.2
  • ICD-11: EC32
  • OMIM: -
  • UMLS: C0079294
  • MeSH: D016108
  • GARD: 2150
  • MedDRA: -

Detailed information

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