Caputo Ruggero, Marzano Angelo Valerio, Passoni Emanuela, Berti Emilio
Institute of Dermatological Sciences, University of Milan-Fondazione IRCCS Ospedale Maggiore Policlinico, Mangiagalli e Regina Elena, Milan, Italy.
J Am Acad Dermatol. 2007 Dec;57(6):1031-45. doi: 10.1016/j.jaad.2007.03.014. Epub 2007 May 7.
Histiocytic syndromes represent a large, heterogeneous group of diseases resulting from proliferation of histiocytes. In addition to the classic variants, the subset of non-Langerhans cell histiocytoses comprises rare entities that have more recently been described. These last include both forms that affect only the skin or the skin and mucous membranes, and usually show a benign clinical behavior, and forms involving also internal organs, which may follow an aggressive course. The goal of this review is to outline the clinical, histologic, and ultrastructural features and the course, prognosis, and management of these unusual histiocytic syndromes.
组织细胞增多症综合征是一组由组织细胞增殖引起的庞大且异质性的疾病。除了经典的变异型外，非朗格汉斯细胞组织细胞增多症亚组还包括一些最近才被描述的罕见疾病。后者既包括仅累及皮肤或皮肤及黏膜的类型，通常表现为良性临床行为，也包括累及内脏器官的类型，其病情可能进展迅速。本综述的目的是概述这些不常见组织细胞增多症综合征的临床、组织学和超微结构特征以及病程、预后和治疗方法。
