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一个同时患有幼年黄色肉芽肿和朗格汉斯细胞组织细胞增生症的孩子。

A child with coexistent juvenile xanthogranuloma and Langerhans cell histiocytosis.

机构信息

Department of Dermatology, Shanghai Jiaotong University Medical School, Xinhua Hospital, Shanghai, China.

出版信息

J Am Acad Dermatol. 2010 Feb;62(2):329-32. doi: 10.1016/j.jaad.2009.02.028. Epub 2009 Dec 6.

DOI:10.1016/j.jaad.2009.02.028
PMID:19969387
Abstract

A 15-month-old boy was seen because of two distinct types of lesions, namely, yellowish papules on the scalp and face, and hemorrhagic macules and papules on the trunk. A biopsy specimen from one of the yellowish papules showed histopathologic and immunohistochemical changes of both juvenile xanthogranuloma and Langerhans cell histiocytosis. The section from the center of the biopsy specimen showed a proliferation of foamy histiocytes, among them Touton giant cells, which were positive for CD68, but negative for S-100 and CD1a. At the edges of the specimen was a predominantly histiocytic infiltration in the papillary dermis that was positive for S-100 and CD1a, but negative for CD68. The patient died 12 days after hospital admission consequent to disseminated intravascular coagulopathy. We did not biopsy the hemorrhagic lesions; however, this combination of findings suggests a possible relationship between juvenile xanthogranuloma and Langerhans cell histiocytosis, as previously reported.

摘要

一名 15 个月大的男童因两种不同类型的皮损就诊,即头皮和面部的黄色丘疹,以及躯干的瘀点和丘疹。从一个黄色丘疹切取的活检标本显示了幼年黄色肉芽肿和朗格汉斯细胞组织细胞增生症的组织病理学和免疫组织化学改变。活检标本中心的切片显示了泡沫状组织细胞的增生,其中包括朗格汉斯巨细胞,这些细胞对 CD68 呈阳性,但对 S-100 和 CD1a 呈阴性。在标本边缘,在乳头真皮中主要是组织细胞浸润,对 S-100 和 CD1a 呈阳性,但对 CD68 呈阴性。患儿在入院后 12 天因弥散性血管内凝血死亡。我们未对出血性皮损进行活检;然而,如先前报道的,这种发现组合提示幼年黄色肉芽肿和朗格汉斯细胞组织细胞增生症之间可能存在关联。

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