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毛囊性蕈样肉芽肿伴大细胞转化,表现为头皮穿掘性蜂窝织炎。

Folliculotropic mycosis fungoides with large-cell transformation presenting as dissecting cellulitis of the scalp.

作者信息

Gilliam A C, Lessin S R, Wilson D M, Salhany K E

机构信息

Department of Dermatology, Case Western Reserve University, U.H.C. Cleveland, OH 44106, USA.

出版信息

J Cutan Pathol. 1997 Mar;24(3):169-75. doi: 10.1111/j.1600-0560.1997.tb01572.x.

Abstract

Follicular mycosis fungoides (MF) is a rare variant of cutaneous T-cell lymphoma (CTCL) in which malignant lymphocytes preferentially infiltrate hair follicles. This report describes a patient with follicular mycosis fungoides presenting in a manner similar to dissecting cellulitis of the scalp with nonhealing, draining nodular lesions. Follicular mucinosis associated with folliculotropic mycosis fungoides resulted in follicular disruption and deep dissecting cellulitis. Large-cell transformation of CTCL was present in the initial diagnostic scalp and axillary lymph node specimens. The patient died from progressive CTCL 9 months following initial diagnosis despite electron beam radiation, topical mechlorethamine, interferon-alpha, and systemic chemotherapy. This case indicates that large-cell transformation of folliculotropic mycosis fungoides is an aggressive form of CTCL, and that folliculotropic mycosis fungoides can give rise to lesions which resemble dissecting cellulitis of the scalp. Upregulation of intercellular adhesion molecule-1 (ICAM-1) on follicular epithelium adjacent to lymphocyte function-associated antigen-1 (LFA-1)-positive folliculotropic lymphoma cells in this report provides insight into lymphocyte homing mechanisms in folliculotropic MF.

摘要

毛囊性蕈样肉芽肿(MF)是皮肤T细胞淋巴瘤(CTCL)的一种罕见变体,其中恶性淋巴细胞优先浸润毛囊。本报告描述了一名毛囊性蕈样肉芽肿患者,其表现类似于头皮穿掘性蜂窝织炎,有不愈合的引流性结节性病变。与亲毛囊性蕈样肉芽肿相关的毛囊黏蛋白沉积导致毛囊破坏和深部穿掘性蜂窝织炎。在最初诊断的头皮和腋窝淋巴结标本中存在CTCL的大细胞转化。尽管进行了电子束放疗、外用氮芥、α干扰素和全身化疗,但患者在初次诊断后9个月死于进行性CTCL。该病例表明,亲毛囊性蕈样肉芽肿的大细胞转化是CTCL的一种侵袭性形式,并且亲毛囊性蕈样肉芽肿可引起类似于头皮穿掘性蜂窝织炎的病变。本报告中,与淋巴细胞功能相关抗原-1(LFA-1)阳性的亲毛囊性淋巴瘤细胞相邻的毛囊上皮细胞间黏附分子-1(ICAM-1)上调,为亲毛囊性MF中的淋巴细胞归巢机制提供了见解。

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