Ectodermose Érosive Pluriorificielleの3例について

Three Cases of Ectodermose Érosive Pluriorificielle

緒言

　Ectodermose　érosive pluriorificielleはRendu （1916），Fiessinger （1917）等が従来のHebra型多形滲出性紅斑とは侵襲部位が異なる独立した新疾患として報告以来，仏国では比較的広く知られた疾患である。

　本疾患の主な症状は，急激な発熱と共に，多数例において皮膚に発疹並びに身体諸開口部粘膜，即ち，眼結膜，口腔，鼻腔，外陰，肛門部等が侵されるものである。

Three cases with persistent sequelae of ec-todermose érosive pluriorificielle are reported. The condition belongs to the so-called muco-cutaneo-ocular syndrome proposed by Robinson (1950).

Case 1 Female, now 28 years of age. At the age of 7 years, sudden fever developed follow-ed, 3 days later, by eruption of the skin re-sembling erythema exsudativa multiforme, ero-sion of the conjunctiva, nasal and oral cavity, pharynx and the vulva as well as shedding of the nails. The acute manifestations subsided leaving trichiasis, diffuse corneal opacity, de-crease of lacrimal secretion and deformity of the nails.