Fischer M, Wohlrab J, Audring T H, Sterry W, Marsch W C
Department of Dermatology and Venerology, Martin-Luther-University Halle-Wittenberg, Halle (Saale), Germany.
J Eur Acad Dermatol Venereol. 2000 May;14(3):196-202. doi: 10.1046/j.1468-3083.2000.00047.x.
Granulomatous mycosis fungoides is an extremely rare type of cutaneous T-cell lymphoma. Two cases are described and checked for clinical, histological and therapeutic differences to other variants of mycosis fungoides.
Case 1: a 52-year-old patient with livid plaques covering the whole integument. Histological findings showed granulomas with multinuclear giant cells in addition to a malignant lymphohistiocytic infiltrate (monodonal T-cell receptor (TCR)-gamma rearrangement). Despite various chemotherapeutic regimens, progression to tumour stage was observed. Case 2: an 88-year-old man with plaques and ulcerating tumours on the trunk and head. Histological findings showed malignant T-lymphocyte infiltrate (monoclonal TCR-gamma rearrangement) and granulomas with multinuclear foreign-body giant cells. Complete regression of all lesions was achieved using both local psoralen-ultraviolet A and electron radiotherapy.
The diagnosis of a granulomatous mycosis fungoides depends exclusively on the histological demonstration of granulomas. Distinct clinical characteristics are not present. Apart from granuloma formation, no other noticeable histological features are evident. The presence of granulomas in mycosis fungoides does not have prognostic implications, as cases with aggressive, but also with a prolonged course have been described.
肉芽肿性蕈样霉菌病是一种极其罕见的皮肤T细胞淋巴瘤类型。本文描述了两例病例,并检查了其与蕈样霉菌病其他变体在临床、组织学和治疗方面的差异。
病例1:一名52岁患者,全身皮肤出现青灰色斑块。组织学检查发现除恶性淋巴组织细胞浸润(单克隆T细胞受体(TCR)-γ重排)外,还有多核巨细胞组成的肉芽肿。尽管采用了各种化疗方案,但仍观察到病情进展至肿瘤期。病例2:一名88岁男性,躯干和头部出现斑块及溃疡性肿瘤。组织学检查发现恶性T淋巴细胞浸润(单克隆TCR-γ重排)以及有多核异物巨细胞的肉芽肿。通过局部补骨脂素-紫外线A光疗和电子放疗,所有病变完全消退。
肉芽肿性蕈样霉菌病的诊断完全依赖于肉芽肿的组织学表现。不存在明显的临床特征。除肉芽肿形成外,无其他明显的组织学特征。蕈样霉菌病中肉芽肿的存在对预后无影响,因为既有侵袭性病程的病例,也有病程迁延的病例被报道过。