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泛发性发疹性组织细胞瘤:一例儿科病例

Generalized eruptive histiocytoma: a pediatric case.

作者信息

Wee S H, Kim H S, Chang S N, Kim D K, Park W H

机构信息

Department of Dermatology, College of Medicine Pochon CHA University, Yonsei University College of Medicine, Seoul, Korea.

出版信息

Pediatr Dermatol. 2000 Nov-Dec;17(6):453-5. doi: 10.1046/j.1525-1470.2000.01842.x.

Abstract

Generalized eruptive histiocytoma (GEH) is a rare, benign, papular, self-healing histiocytosis characterized by recurrent crops of small, firm, tan to reddish papules that appear in a symmetrical fashion on the face, trunk and arms, affecting mainly adults. Approximately 31 cases have been described, of which 8 were in children. A new concept unifying the confusing potpourri of non-Langerhans cell histiocytosis has recently been described in which GEH has been considered to represent an early undifferentiated stage of various histiocytic disorders. We describe a 9-year-old boy who had all the features of GEH and emphasize the importance of clinical, histologic, immunohistochemical, and ultrastructural examination in the diagnosis of histiocytic disorders. On the basis of the increasing numbers of similar reported cases showing overlap in clinical and histologic features and in accordance with the new unifying concept of non-Langerhans cell histiocytosis, we presume that this group of disorders may represent a continuous spectrum of a single disease rather than a collection of separate disorders.

摘要

泛发性发疹性组织细胞瘤(GEH)是一种罕见的良性丘疹性自愈性组织细胞增多症,其特征为反复成批出现小而坚实的棕褐色至红色丘疹,呈对称分布于面部、躯干和手臂,主要累及成年人。大约已报道31例,其中8例为儿童。最近描述了一个新的概念,将非朗格汉斯细胞组织细胞增多症令人困惑的混杂情况统一起来,其中GEH被认为代表各种组织细胞疾病的早期未分化阶段。我们描述了一名具有GEH所有特征的9岁男孩,并强调临床、组织学、免疫组织化学和超微结构检查在组织细胞疾病诊断中的重要性。基于越来越多报道的类似病例在临床和组织学特征上存在重叠,并且根据非朗格汉斯细胞组织细胞增多症的新统一概念,我们推测这组疾病可能代表单一疾病的连续谱系,而非单独疾病的集合。

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