Two Cases of Anti-p200 Pemphigoid / 대한피부과학회지

Anti-p200 pemphigoid is a recently defined subepidermal immunobullous disease. Most anti-p200 pemphigoid patients show the clinical features of bullous pemphigoid, dermatitis herpetiformis and linear IgA disease. It is characterized by the binding of circulating IgG autoantibodies to the dermal side of 1 M NaCl split skin, and by reactivity of these autoantibodies to a unique 200-kD antigen on immunoblot of dermal extract. On immunoelectron microscopic examination, these autoantibodies deposit at the lamina lucida-lamina densa interface. Herein, we report two cases of anti-p200 pemphigoid, as well as their favorable response to treatment with systemic corticosteroid, colchicine and dapsone.

A Korean Case of Anti-p200 Pemphigoid

Anti-p200 pemphigoid is a newly defined autoimmune subepidermal blistering disease, which is characterized by the presence of IgG autoantibodies to the dermal side of 1M NaCl split skin as well as by the reactivity of these antibodies to a novel 200-kDa antigen on immunoblot analysis of a dermal extract. We describe a 49-year-old Korean male who presented with a bullous eruption on the whole body, which clinically resembled bullous pemphigoid or epidermolysis bullosa acquisita. A histopathological examination of a lesional skin biopsy specimen showed an area of dermal-epidermal separation and mixed dermal inflammatory infiltrates consisting of lymphocytes, neutrophils, and eosinophils. Direct immunofluorescence showed a linear deposition of IgG and C3 along the basement membrane zone. Indirect immunofluorescence demonstrated circulating IgG autoantibodies directed against the dermal side of the 1M NaCl split skin. Immunoblot analysis of dermal extracts revealed the patient's sera recognized the 200-kDa antigen. This is the first Korean case of an anti-p200 pemphigoid who showed good response to the treatment with systemic corticosteroids and dapsone.