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两例隐性营养不良型大疱性表皮松解症婴儿的非典型黑素细胞病变

Two cases of atypical melanocytic lesions in recessive dystrophic epidermolysis bullosa infants.

作者信息

Natsuga K, Akiyama M, Sato-Matsumura K C, Tsuchiya K, Shimizu H

机构信息

Department of Dermatology, Hokkaido University Graduate School of Medicine, and Department of Dermatology, Sapporo City General Hospital, Sapporo, Japan.

出版信息

Clin Exp Dermatol. 2005 Nov;30(6):636-9. doi: 10.1111/j.1365-2230.2005.01822.x.

DOI:10.1111/j.1365-2230.2005.01822.x
PMID:16197375
Abstract

Summary Atypical melanocytic lesions (AtML) are known to be associated with epidermolysis bullosa (EB), mainly with the junctional subtype. We report two cases of AtML in two female infants with recessive dystrophic epidermolysis bullosa (RDEB). Both lesions were dark brown- to black-coloured, asymmetric-shaped macules, 3-4 cm in size, with an irregular border and were located on the forearms of two unrelated, 1-year-old female infants. On a clinical and pathological basis, the pigmented macules were diagnosed as AtML in EB patients. There are only a few reports describing in detail the clinical and histopathological features of AtML in RDEB, especially in infant cases. AtML may easily be misdiagnosed as malignant melanoma and, even in infant patients with RDEB, this should be included as one of the differential diagnosis of pigmented lesions.

摘要

摘要 已知非典型黑素细胞病变(AtML)与大疱性表皮松解症(EB)相关,主要与交界型亚型有关。我们报告了两例患有隐性营养不良性大疱性表皮松解症(RDEB)的女婴发生AtML的病例。两处病变均为深褐色至黑色、不对称的斑疹,大小为3 - 4厘米,边界不规则,位于两名无亲缘关系的1岁女婴的前臂。基于临床和病理检查,这些色素沉着斑疹在EB患者中被诊断为AtML。仅有少数报告详细描述了RDEB中AtML的临床和组织病理学特征,尤其是婴儿病例。AtML很容易被误诊为恶性黑色素瘤,即使在患有RDEB的婴儿患者中,这也应作为色素沉着病变的鉴别诊断之一。

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