Anti-p200 pemphigoid
ORPHA:454710· ICD-10 L12.8
Definition
A rare, acquired, subepidermal autoimmune bullous disease characterized by polymorphic cutaneous lesions (blisters, urticarial lesions or scars/milia) associated with imunoglubulin G deposition in the basement membrane zone. Lesions are frequently localized on extremities, trunk, palmoplantar and cephalic areas as well as mucous membranes.
- Prevalence
- <1 / 1 000 000
- Inheritance
- Not applicable
- Age of onset
- Adult, Elderly