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进行性黄斑部色素减退症

Progressive macular hypomelanosis.

作者信息

Elmariah Sarina B, Kundu Roopal V

机构信息

Department of Dermatology, Massachusetts General Hospital, Boston, MA, USA.

出版信息

J Drugs Dermatol. 2011 May;10(5):502-6.

Abstract

Progressive macular hypomelanosis is an under-recognized disorder characterized by the presence of numerous ill-defined hypopigmented macules and patches on the trunk of young adults. Although common, particularly in Fitzpatrick skin types IV-VI, this condition is frequently misdiagnosed and treated inadequately with antifungals or topical steroids resulting in patient frustration. The exact pathogenesis of progressive macular hypomelanosis is unknown; however, recent studies suggest hypopigmentation results from decreased melanin formation and altered melanosome distribution in response to Proprionibacterium. While there are no well-established or consistently effective therapies for progressive macular hypomelanosis, our growing understanding of its pathogenesis urges consideration of alternative treatment strategies. Here, we report five patients with progressive macular hypomelanosis who benefitted from topical and systemic antimicrobial therapy and summarize the current clinical, pathological and treatment paradigms of this disorder.

摘要

进行性黄斑部色素减退症是一种未得到充分认识的疾病,其特征为在年轻成年人的躯干上出现众多边界不清的色素减退斑和斑片。尽管这种疾病很常见,尤其是在菲茨帕特里克皮肤分型为IV - VI型的人群中,但它经常被误诊,并用抗真菌药或外用类固醇进行不充分的治疗,从而导致患者感到沮丧。进行性黄斑部色素减退症的确切发病机制尚不清楚;然而,最近的研究表明,色素减退是由于黑色素生成减少以及对丙酸杆菌产生反应时黑素小体分布改变所致。虽然目前尚无针对进行性黄斑部色素减退症的确立完善或始终有效的治疗方法,但我们对其发病机制的认识不断增加,促使我们考虑采用替代治疗策略。在此,我们报告5例受益于局部和全身抗菌治疗的进行性黄斑部色素减退症患者,并总结该疾病目前的临床、病理和治疗模式。

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