Orphanet: Dermatitis herpetiformis

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Dermatitis herpetiformis

Disease definition

A chronic autoimmune subepidermal bullous disease characterized by grouped pruritic lesions such as papules, urticarial plaques, erythema, and herpetiform vesiculae, with a predominantly symmetrical distribution on extensor surfaces of the elbows (90%), knees (30%), shoulders, buttocks, sacral region, and face of children and adults. Erosions, excoriations and hyperpigmentation usually follow. It may also appear as a consequence of gluten intolerance.


Classification level: Disorder
  • Synonym(s):
    • Duhring-Brocq disease
  • Prevalence: 1-5 / 10 000
  • Inheritance: Not applicable 
  • Age of onset: All ages
  • ICD-10: L13.0
  • OMIM: 601230
  • UMLS: C0011608
  • MeSH: D003874
  • GARD: 1917
  • MedDRA: 10012468

Detailed information


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