Catalog

Cicatricial pemphigoid, Brunsting-Perry type

No MMS code

Link to WHO Definition

Cicatricial pemphigoid, Brunsting-Perry type – http://id.who.int/icd/entity/1148757980

A rare scarring immunobullous skin disorder due to autoantibodies to components of the epidermal basement membrane, particularly the anchoring filament component laminin 5 and the hemidesmosome-associated proteins BP180 and BP230. A proportion of patients can be shown to have antibodies to collagen VII and are therefore considered to have a variant of epidermolysis bullosa acquisita (Epidermolysis bullosa acquisita: Brunsting-Perry type).