类固醇硫酸酯酶缺乏性鱼鳞病。76例临床研究。

Ichthyosis of steroid sulphatase deficiency. Clinical study of 76 cases.

作者信息

Høyer H, Lykkesfeldt G, Ibsen H H, Brandrup F

出版信息

Dermatologica. 1986;172(4):184-90. doi: 10.1159/000249332.

Abstract

A detailed clinical study of 76 males with steroid sulphatase deficiency confirmed the ichthyosis of this disorder to be identical with the classical descriptions of recessive X-linked ichthyosis. The appearance of regular ichthyotic scaling seems in most instances to be heralded by a general peeling of the skin. This characteristic feature was registered between age 1 and 3 weeks in 19 of 21 young boys followed due to a placental steroid sulphatase deficiency. Later on, polygonal scales giving a 'dirty' appearance were most pronounced on the distal parts of the body and extensor aspects of the extremities. The ichthyosis aggravates through childhood into adult age.

摘要

一项对76名类固醇硫酸酯酶缺乏症男性患者的详细临床研究证实，该疾病的鱼鳞病与隐性X连锁鱼鳞病的经典描述完全相同。在大多数情况下，规则的鱼鳞病鳞屑出现之前，皮肤通常会先出现普遍的脱皮现象。在21名因胎盘类固醇硫酸酯酶缺乏而接受随访的男童中，有19名在1至3周龄时出现了这一特征性表现。随后，身体远端部位和四肢伸侧出现的呈“脏污”外观的多边形鳞屑最为明显。鱼鳞病在儿童期到成年期会逐渐加重。