Trüeb R M, Meyer J C
Department of Dermatology, University Hospital of Zurich, Switzerland.
Dermatology. 2000;200(3):247-9. doi: 10.1159/000018368.
X-linked recessive ichthyosis (XRI) is a genetic disorder of keratinization with extracutaneous manifestations due to deficiency of steroid sulfatase (STS). Because STS plays an important role in androgen metabolism, and elevated levels of dehydroepiandrosterone sulfate have been reported in young men with andogenetic alopecia (AGA), the hypothesis was advanced that men with XRI do not show AGA or develop only mild forms of common baldness.
Patients with a diagnosis of XRI confirmed by analysis of the microsomal sulfatases in our clinic between 1984 and 1998, and in whom study of the case histories depicted the typical clinical presentation of XRI, were sent a questionnaire with the Hamilton-Norwood scale of patterns of hair loss in men, inquiring them to designate the condition of their scalp hair.
Of 15 questionnaires returned, 7 indicated Hamilton-Norwood I, 3 Hamilton-Norwood II, 3 Hamilton-Norwood III-vertex, 1 Hamilton-Norwood IV and 1 Hamilton-Norwood VII. The results of this survey do not support the hypothesis that XRI and AGA are mutually exclusive, in as much as advanced AGA was found among these men.
X连锁隐性鱼鳞病（XRI）是一种由于类固醇硫酸酯酶（STS）缺乏导致的伴有皮肤外表现的角化遗传性疾病。由于STS在雄激素代谢中起重要作用，并且有报道称早秃（AGA）青年男性硫酸脱氢表雄酮水平升高，因此有人提出假说，即患有XRI的男性不会出现AGA或仅发展为轻度常见秃发形式。
1984年至1998年间在我们诊所通过微粒体硫酸酯酶分析确诊为XRI且病历研究显示有典型XRI临床表现的患者，收到了一份附有男性脱发汉密尔顿-诺伍德分级量表的问卷，询问他们头皮头发的状况。
在回收的15份问卷中，7份显示为汉密尔顿-诺伍德I级，3份为汉密尔顿-诺伍德II级，3份为汉密尔顿-诺伍德III级（头顶型），1份为汉密尔顿-诺伍德IV级，1份为汉密尔顿-诺伍德VII级。这项调查结果不支持XRI和AGA相互排斥的假说，因为在这些男性中发现了重度AGA。
