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面部皮肤剥脱综合征:一例病例报告及简要综述。

Facial peeling skin syndrome: a case report and a brief review.

作者信息

Janjua Shahbaz Ahmad, Hussain Ijaz, Khachemoune Amor

机构信息

Ayza Skin and Research Center, Lalamusa, Pakistan.

出版信息

Int J Dermatol. 2007 Mar;46(3):287-9. doi: 10.1111/j.1365-4632.2006.03074.x.

Abstract

Peeling skin syndrome is an extremely rare genodermatosis of possible autosomal recessive inheritance, characterized by asymptomatic spontaneous exfoliation of the stratum corneum at a subcorneal or intracorneal level. It usually presents at birth or appears later in early childhood. The condition may be generalized or localized. Here we describe a case of localized continual skin peeling limited to the facial skin in a 6-month-old infant, with two other members of the family affected with the same condition. A few cases of localized skin peeling limited to the acral surfaces have been described in the literature, but a familial case of localized skin peeling limited to the facial skin has not been described before. We believe that our patient represents a new subtype of peeling skin syndrome, limited to the skin of the face.

摘要

皮肤剥脱综合征是一种极为罕见的遗传性皮肤病,可能为常染色体隐性遗传,其特征是角质层在角膜下或角膜内水平出现无症状的自发性剥脱。它通常在出生时出现或在幼儿期早期出现。病情可能是全身性的或局限性的。在此,我们描述一例6个月大婴儿局限性持续性皮肤剥脱病例,该剥脱仅限于面部皮肤,且家族中的另外两名成员也患有相同病症。文献中曾描述过几例仅限于肢端表面的局限性皮肤剥脱病例,但此前尚未有过仅限于面部皮肤的家族性局限性皮肤剥脱病例的报道。我们认为,我们的患者代表了一种仅限于面部皮肤的皮肤剥脱综合征新亚型。

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