LeBoit P E, Zackheim H S, White C R
Department of Pathology, School of Medicine, University of California, San Francisco 94143.
Am J Surg Pathol. 1988 Feb;12(2):83-95. doi: 10.1097/00000478-198802000-00002.
Granulomatous mycosis fungoides is an unusual histologic variant of mycosis fungoides, a condition that is ordinarily indolent. Granulomatous slack skin, like granulomatous mycosis fungoides, shows epidermotropism, granulomatous inflammation, a clonal T-helper cell population, and progression to systemic lymphoma in some cases. Unlike granulomatous mycosis fungoides, it is characterized clinically by bulky, pendulous skin folds. The similarities between the two conditions prompted us to compare the histologic features. We reviewed 24 biopsies from 10 patients with granulomatous mycosis fungoides. These showed several distinct histologic patterns, including three cases that mimicked granuloma annulare. We also reviewed biopsy specimens from four patients with granulomatous slack skin. These specimens had a more stereotypic appearance, with permeation of the entire dermis and subcutis by lymphocytes, marked epidermotropism, and a more even distribution of granulomas and giant cells within the infiltrate. Biopsies of fully developed lesions of granulomatous slack skin showed elastolysis involving the full thickness of the dermis--a feature not seen in any of our granulomatous mycosis fungoides cases. Biopsy specimens from granulomatous mycosis fungoides and granulomatous slack skin may be mistaken for nonneoplastic granulomatous dermatitides, but they can usually be distinguished from these by the presence of epidermotropism or atypical lymphocytes. Because several of our patients with granulomatous mycosis fungoides died after courses of unremarkable length, it seems unlikely that the presence of granulomas is invariably correlated with a more benign course than nongranulomatous mycosis fungoides.
肉芽肿性蕈样霉菌病是蕈样霉菌病一种不常见的组织学变异型,蕈样霉菌病通常病程缓慢。与肉芽肿性蕈样霉菌病一样,肉芽肿性皮肤松弛症表现为亲表皮性、肉芽肿性炎症、克隆性辅助性T细胞群,且在某些情况下会进展为系统性淋巴瘤。与肉芽肿性蕈样霉菌病不同,其临床特征为皮肤褶皱粗大、下垂。这两种疾病的相似之处促使我们比较它们的组织学特征。我们回顾了10例肉芽肿性蕈样霉菌病患者的24份活检标本。这些标本呈现出几种不同的组织学模式,包括3例类似环状肉芽肿的病例。我们还回顾了4例肉芽肿性皮肤松弛症患者的活检标本。这些标本外观更具特征性,淋巴细胞弥漫浸润整个真皮和皮下组织,有明显的亲表皮性,且浸润内肉芽肿和巨细胞分布更均匀。肉芽肿性皮肤松弛症完全发展病变的活检显示弹性组织离解累及真皮全层,这一特征在我们所有肉芽肿性蕈样霉菌病病例中均未见到。肉芽肿性蕈样霉菌病和肉芽肿性皮肤松弛症的活检标本可能会被误诊为非肿瘤性肉芽肿性皮炎,但通常可通过亲表皮性或非典型淋巴细胞的存在将它们与非肿瘤性肉芽肿性皮炎区分开来。由于我们的几位肉芽肿性蕈样霉菌病患者在病程并无异常的情况下死亡,因此肉芽肿的存在似乎并不总是与比非肉芽肿性蕈样霉菌病更良性的病程相关。