4世代にわたり慢性円板状エリテマトーデス様皮疹を発生した一家系について

FAMILIAR OCCURRENCE OF A DISCOID LUPUS ERYTHEMATOSUS-LIKE ERUPTION IN FOUR GENERATIONS

はじめに

　皮膚疾患において，家系内発生をみ，遺伝性が問題となつている疾患には多くのものがある。尋常性魚鱗癬，遺伝性掌蹠角化症，ダリエー病，先天性表皮形成不全症，雀卵斑，色素性乾皮症などいく多のものが数えられるが，最近著者らは，顔面，耳殻，四肢の露出部に紅斑，角化，萎縮の三主症状をそなえ，夏季軽快，冬季悪化を幼児期よりくり返している皮疹を，4世代にわたつて発症している一家系を経験した。この家系にみられた皮疹の性状，共通点などを紹介するとともに，この疾患が包含する問題点について論じてみたい。

A family with a discoid l.e.-like eruption in 4 generations, which had 6 patients of 15 members, was reported. All cases showed a discoid l.e.-like eruption over the exposed areas, that is the faces, hands, feet, knees, and ear-lobes, which became worse in winter and better in summer. Some of the nails were deformed or fell off. While disturbance of movement of fingers and toes was noted, the roentgenograms failed to prove defects of their bones.

Onsets of the disease were within one year of age, and the paients were rather short of sta-Laboratory tests showed negative in tuberculin test, LE phenomenon and chromosome abnorma-lity, but positive in RA reaction, hypergammaglobulinemia and increased e.s.r..

Histologic specimens proved marked hyperkeratosis with parakeratosis but no liquefaction degeneration of the basal cell layer. Characteristic band-like lymphocytic infiltration and dilatation of the blood vessels were noted. The hyaline degeneration in some parts of the epidermis and hyaline droplets depositions in the upper parts of the dermis were proved.

They were diagnosed as a type of chilblain lupus.