Pseudoxanthoma elasticumに併発したElastosis perforans serpiginosa

ELASTOSIS PERFORANS SERPIGINOSA ASSOCIATED WITH PSEUDOXANTHOMA ELASTICUM

I．緒言

　1953年Lutz^1）により人体の関節周辺皮膚，特に頸部に好発する環状ないし蛇行状配列を示す赤褐色角化性丘疹で，組織学的に表皮を穿孔する真皮弾力線維組織を特徴とする疾患がKeratosisfollicularis serpiginosaとして報告されて以来，同様の症例が内外に諸家により種々の名称で報告されているが，1958年Dammert-Putkonen^2）の提唱したElastosis perforans serpiginosa （EPS）が診断名として近年多く採られている。この間，本症の合併症，発生機序および共通する変性物質の検索などに関して多数の論著に接するが，このたび，著者は30歳女性のPseudoxanthoma ela-sticum （PXE）に併発したEPSを観察する機会を得たので，2,3文献的考察を加えて報告する。

A 30-year-old housewise has had typical pseudoxanthoma elasticum on the sideneck, axillar and hypogastrial regions since 16 years of age. She also has noticed reddish brown, keratotic papules in circular or archform arrangement and worm-eaten atrophic scars on the central part of bilateral sideneck since 25 years of age. Her past and familiar history had nothing contributive except that her parents were of consanguinity.

Histologic section of pseudoxanthoma elasticum was typical. That from keratotic papule showed giant follicular horny plugs surrounded by acanthotic epidermis, the bottom of which was open to the reticular layer of the dermis.

The channel contained cellular debris of inflammatory infiltrates, amorphous basophilic meterial stained positive by Weigert's stain and the one stained positive by Kossa's stain. Around the opening of the channel in the dermis epitheloid cells, foreign body giant cells and inflammatory infiltrates were seen.

Ophthalmologically angioid streaks of the fundi were revealed. Serum vitamin A level was low (87.3 Iu/dl).

Skin manifestations were markedly improved by long term administration of high doses of vitamin E. Vitamin A deficiency might be one of the factors accelerating deposition of acid mucopolysaccharide of connective tissue in this disease.