Cicatricial pemphigoid, Brunsting-Perry type (Q104444)

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).