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蕈样肉芽肿伴不寻常的表皮增生。

Mycosis fungoides associated with unusual epidermal hyperplasia.

作者信息

Puig L, Musulén E, Fernández-Figueras M T, Miralles J, Sitjas D, De Moragas J M

机构信息

Department of Dermatology, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain.

出版信息

Clin Exp Dermatol. 1996 Jan;21(1):61-4.

PMID:8689775
Abstract

A 58-year-old white woman presented with widespread pruritic brownish plaques and hyperpigmented flexural lesions with a velvety appearance. On histopathological examination, the macules were diagnostic of mycosis fungoides, plaque stage, and the flexural lesions showed epidermal hyperplasia with a seborrhoeic keratosis-like appearance. There was intense mucin deposition and marked reduction of elastic fibres in the papillary dermis, as well as a moderately dense dermal lymphoid infiltrate composed of CD4+ T cells with occasional atypia and focal epidermotropism. The clinical and pathological manifestations of cutaneous T-cell lymphomas, including mycosis fungoides (MF), may show considerable variation. Apart from the three classic stages, a number of unusual clinical presentations and a broad spectrum of histopathological findings have been reported in the literature. In this report, a case of MF, plaque stage, with flexural lesions clinically reminiscent of (pseudo)acanthosis nigricans is presented. On histopathological examination, there was epidermal hyperplasia with a seborrhoeic keratosis-like appearance, with intense deposition of mucin and marked reduction of elastic fibres in the papillary dermis, together with a lymphoid infiltrate composed mainly of CD4+ cells with occasional atypia and epidermotropism. Cytokines produced by the lymphocytes in the infiltrate might account for the epithelial and dermal changes that characterize this peculiar variant of MF.

摘要

一名58岁的白人女性,全身出现瘙痒性褐色斑块以及外观呈天鹅绒样的屈侧色素沉着性损害。组织病理学检查显示,斑疹诊断为蕈样肉芽肿斑块期,屈侧损害表现为表皮增生,呈脂溢性角化病样外观。乳头真皮层有大量黏蛋白沉积和弹性纤维显著减少,还有由CD4 + T细胞组成的中度致密的真皮淋巴样浸润,偶见异型性及灶状亲表皮现象。皮肤T细胞淋巴瘤,包括蕈样肉芽肿(MF)的临床和病理表现可能有很大差异。除了三个经典阶段外,文献中还报道了许多不寻常的临床表现和广泛的组织病理学发现。在本报告中,呈现了1例蕈样肉芽肿斑块期病例,其屈侧损害临床上类似(假性)黑棘皮病。组织病理学检查显示,表皮增生呈脂溢性角化病样外观,乳头真皮层有大量黏蛋白沉积和弹性纤维显著减少,同时有主要由CD4 +细胞组成的淋巴样浸润,偶见异型性和亲表皮现象。浸润中的淋巴细胞产生的细胞因子可能是导致这种特殊类型MF特征性上皮和真皮改变的原因。

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