We describe a Japanese man with antiepiligrin cicatricial pemphigoid and typical clinical features, including ocular involvement. Direct immunofluorescence showed IgG deposition at the basement membrane zone. Indirect immunofluorescence of IM sodium chloride-split skin showed circulating antibasement membrane zone antibodies of IgG class reactive with the dermal side of the split. Immunoblotting of human epidermal and dermal extracts, as well as a bacterial fusion protein of BP180 NC16a domain, showed no specific reactivity. In contrast, with immunoprecipitation of either culture medium or cell lysate from normal keratinocytes, the patient s serum clearly reacted with the protein epiligrin, a laminin isoform present in the lamina lucida of the human epidermal basement membrane zone. This is the first confirmed case of a Japanese patient with this disease entity.
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