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毛囊黏蛋白沉积性亲毛囊性蕈样肉芽肿——病例报告

Folliculotropic mycosis fungoides with follicular mucinosis--case report.

作者信息

Rados Jaka, Dobrić Ivan, Pasić Aida, Loncarić Davorin, Manola Ivana

机构信息

University Department of Dermatology and Venereology, Zagreb University Hospital Center, Salata 4 HR-10000 Zagreb, Croatia.

出版信息

Acta Dermatovenerol Croat. 2006;14(2):94-9.

Abstract

Reports on clinical and histologic follicular alterations in patients previously diagnosed with mycosis fungoides (MF) or at the time of MF diagnosis are rare. The clinical and histologic criteria to distinguish MF associated with follicular mucinosis from follicular MF are a matter of debate. A patient is described with advanced clinical and histologic alterations predominated by follicular lesions and presence of mucin. In the early stage of the disease, folliculotropism was clinically and histologically present but less pronounced than epidermotropism and classic plaque-like lesions. The patient died four years after the diagnosis. As the term 'folliculotropic' describes a particular histopathologic finding, we consider it correct to use the term "folliculotropic MF" to denote atypical lymphocyte folliculotropism in the absence or presence of mild epidermotropism, presence of mucin, or no evidence for intrafollicular mucin. Folliculotropic MF seems to represent a specific clinicopathologic entity which may have a poorer prognosis than classic MF.

摘要

关于先前诊断为蕈样肉芽肿(MF)的患者或在MF诊断时的临床和组织学滤泡改变的报道很少。区分与滤泡性粘蛋白沉积症相关的MF和滤泡性MF的临床和组织学标准存在争议。本文描述了一名患者,其临床和组织学改变进展,以滤泡性病变和粘蛋白的存在为主。在疾病早期,临床上和组织学上均存在向滤泡性生长,但不如向表皮性生长和典型斑块样病变明显。该患者在诊断后四年死亡。由于“向滤泡性生长”描述了一种特定的组织病理学发现,我们认为使用“向滤泡性生长的MF”一词来表示在不存在或存在轻度向表皮性生长、存在粘蛋白或无滤泡内粘蛋白证据的情况下非典型淋巴细胞向滤泡性生长是正确的。向滤泡性生长的MF似乎代表一种特定的临床病理实体,其预后可能比经典MF更差。

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