Antiepiligrin cicatricial pemphigoid: the first case report from Japan

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 1M 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.