Newman Brenda, Hu Weimin, Nigro Kelly, Gilliam Anita C
Department of Dermatology, Case/University Hospitals of Cleveland, Ohio 44106-5028, USA.
J Am Acad Dermatol. 2007 Feb;56(2):302-16. doi: 10.1016/j.jaad.2006.06.010. Epub 2006 Oct 9.
Histiocytoses are a heterogeneous group of disorders that are characterized by the proliferation and accumulation of reactive or neoplastic histiocytes. Three classes of histiocytoses have been defined: class I, Langerhans cell disease; class II, non-Langerhans cell histiocytic disease without features of malignancy; and class III, malignant histiocytic disorders. Although the disorders in classes I and II usually have a benign appearance on histology and are commonly non-aggressive and self-healing, some can cause debilitating or even fatal outcomes. Such cases beg the question: what stimulates aggressive behavior of a classically benign disease? New molecular information may now provide insight into the driving force behind many of the aggressive histiocytoses. In this article, we review Langerhans cell disease and seven aggressive histiocytoses that can involve skin, discuss histologic features that may forecast a poor prognosis, and discuss the molecular findings that help to explain the pathophysiology of these aggressive histiocytic disorders.
组织细胞增多症是一组异质性疾病，其特征为反应性或肿瘤性组织细胞的增殖和积聚。已定义了三类组织细胞增多症：I类，朗格汉斯细胞病；II类，无恶性特征的非朗格汉斯细胞组织细胞病；III类，恶性组织细胞疾病。尽管I类和II类疾病在组织学上通常表现为良性，通常不具侵袭性且可自愈，但有些疾病可能导致衰弱甚至致命的后果。此类病例引发了一个问题：是什么刺激了一种典型良性疾病的侵袭性表现？新的分子信息现在可能为许多侵袭性组织细胞增多症背后的驱动因素提供见解。在本文中，我们回顾了朗格汉斯细胞病以及七种可能累及皮肤的侵袭性组织细胞增多症，讨论了可能预示预后不良的组织学特征，并讨论了有助于解释这些侵袭性组织细胞疾病病理生理学的分子研究结果。
