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 1 M 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 1 M 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.