Anti-p200 Pemphigoid / 대한피부과학회지

ABSTRACT

Anti-p200 pemphigoid is an autoimmune subepidermal bullous disease characterized by circulating and tissue-bound autoantibodies directed against a 200 kD protein of the human dermis. We report a 78-year-old male who was presented with bullous eruptions on the trunk, extremities, which clinically resemble bullous pemphigoid, epidermolysis bullosa aquisita, linear IgA dermatosis or dermatitis herpetiformis. Oral muscosa of the lower lip was also affected. Histopathological examination of a skin biopsy specimen from the trunk revealed subepidermal blister and infiltration predominantly by neutrophils. Direct immunofluorescence revealed linear deposits of IgG, C3 and IgA at the basement membrane zone. Indirect immunofluorescence using salt-split skin showed that IgG antibodies bound on the dermal side. Immunoblotting with dermal extracts showed that the patient's IgG autoantibodies reacted with a 200 kD protein. The patient showed good response to dapsone.