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[克里奥尔色素沉着异常或吉耶 - 埃勒农混血人种的特发性黄斑部色素减退症]

[Creole dyschromia or idiopathic macular hypomelanosis of the melanodermic halfcast of Guillet-Hélénon].

作者信息

Guillet G, Guillet M H

机构信息

CHU Morvan, Brest.

出版信息

Bull Soc Pathol Exot. 1997;90(5):333-4.

PMID:9507764
Abstract

Melanodermic halfcasts may develop an original cutaneous dyschromia known as "progressive and extensive hypomelanosis" (Guillet-Helenon 1988). This disease is characterized by hypochromic and coalescent macules on the back and abdomen with possible spontaneous improvement within five years, favoured by UV exposure. The disease is not restricted to a limited geographic group: eight observations were collected in melanodermic patients leaving in temperate area. The pathogenesis of the disorder involves a variation in melanosome size and distribution with decrease in production of type IV melanosomes featuring a change of ultrastructural phenotype of melanogenesis. Since it may be misdiagnosed as fungal disease, leprosy or achromic eczema leading to useless laboratory examinations, this specific and frequent disease deserves to be known and recognized.

摘要

黑皮肤人种的半身塑像可能会出现一种原发型皮肤色素异常,称为“进行性广泛性色素减退症”(吉耶 - 海伦农,1988年)。这种疾病的特征是背部和腹部出现色素减退且融合的斑疹,可能在五年内自发改善,紫外线照射对此有促进作用。该疾病并不局限于特定地理区域的人群:在居住于温带地区的黑皮肤患者中收集到了8例病例。该病症的发病机制涉及黑素小体大小和分布的变化,IV型黑素小体生成减少,其特征为黑素生成超微结构表型的改变。由于它可能被误诊为真菌病、麻风病或色素减退性湿疹,从而导致进行无用的实验室检查,这种独特且常见的疾病值得被了解和认识。

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