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Dermatitis herpetiformis

ORPHA:1656· ICD-10 L13.0

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.

Prevalence
1-5 / 10 000
Inheritance
Not applicable
Age of onset
All ages