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先天性自愈性组织细胞增多症。两例病例报告并进行组织化学和超微结构研究

Congenital self-healing histiocytosis. Report of two cases with histochemical and ultrastructural studies.

作者信息

Divaris D X, Ling F C, Prentice R S

机构信息

Department of Pathology, Queen's University, Kingston, Ontario, Canada.

出版信息

Am J Dermatopathol. 1991 Oct;13(5):481-7.

PMID:1951985
Abstract

Congenital self-healing histiocytosis (CSHH) is a rare primary skin disorder. Of the two cases in newborn infants reported here, one had numerous widespread lesions while the other had a solitary ulcerating scalp nodule. Both neonates were otherwise healthy; neither exhibited either systemic involvement or involvement of mucous membranes. The findings drawn from the skin biopsies, including histology, S-100 positivity in the majority of the cells, and the presence of Birbeck granules, were indistinguishable from those described in infantile Letterer-Siwe disease (histiocytosis X). However, the benign clinical course, with rapid regression of the nodules in both cases, was diagnostic of CSHH.

摘要

先天性自愈性组织细胞增多症(CSHH)是一种罕见的原发性皮肤疾病。在此报告的两例新生儿病例中,一例有大量广泛分布的皮损,另一例有一个孤立的溃疡性头皮结节。两名新生儿其他方面均健康;均未表现出系统性受累或黏膜受累。皮肤活检结果,包括组织学、大多数细胞S-100阳性以及存在伯贝克颗粒,与婴儿勒-雪病(组织细胞增多症X)中描述的结果无法区分。然而,两例病例中结节均迅速消退的良性临床过程可诊断为CSHH。

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Congenital self-healing histiocytosis.先天性自愈性组织细胞增多症。
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