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蕈样肉芽肿伴表皮粘蛋白沉积症:一种具有海绵样模式的蕈样肉芽肿变体。

Mycosis fungoides with epidermal mucinosis: a variant of mycosis fungoides with a spongiosis-like pattern.

作者信息

Hu Stephanie W, Ratech Howard, Naeem Rizwan, Latkowski Jo-Ann, Kamino Hideko

机构信息

Dermatopathology Section, Ronald O. Perelman Department of Dermatology, New York University School of Medicine, New York, NY, USA.

Department of Pathology, Montefiore Medical Center, Albert Einstein College of Medicine, Bronx, NY, USA.

出版信息

J Cutan Pathol. 2015 Oct;42(10):730-8. doi: 10.1111/cup.12513. Epub 2015 Jul 3.

Abstract

BACKGROUND

The histopathologic diagnosis of mycosis fungoides (MF) has classically relied on the presence of atypical epidermotropic T-lymphocytes predominating over spongiosis. However, in some cases of MF, prominent epidermal mucinosis in a spongiosis-like pattern mimics a spongiotic dermatitis. To our knowledge, only one series in the literature has thus far recognized the presence of epidermal mucinosis in MF.

METHODS

We evaluated 30 skin biopsies from 18 patients with the clinical diagnosis of MF, which fulfilled all histopathologic criteria for patch- or plaque-stage MF, but also showed epidermal mucinosis in a spongiosis-like pattern. A total of 15 specimens were studied by immunohistochemistry, and seven were tested for T-cell receptor (TCR) gene rearrangements. Twenty biopsies of spongiotic dermatitides were included as controls.

RESULTS

We confirmed the presence of epidermal mucinosis in all 30 cases of MF with a spongiosis-like pattern based on histopathologic criteria and the colloidal iron stain for mucin. Immunohistochemistry in 15 specimens showed significant loss of pan-T-cell antigens CD5 (10/15) and CD7 (14/15); and TCR clonality was detected in 7 specimens from 6 patients, supporting the diagnosis of MF.

CONCLUSIONS

We report helpful histopathologic criteria for distinguishing MF with epidermal mucinosis in a spongiosis-like pattern from spongiotic dermatitis.

摘要

背景

蕈样肉芽肿(MF)的组织病理学诊断传统上依赖于非典型亲表皮性T淋巴细胞的存在且其数量超过海绵形成。然而,在某些MF病例中,呈海绵形成样模式的显著表皮黏蛋白沉积症可模拟海绵状皮炎。据我们所知,迄今为止文献中仅有一个系列报道了MF中存在表皮黏蛋白沉积症。

方法

我们评估了18例临床诊断为MF的患者的30份皮肤活检标本,这些标本符合斑块期或斑片期MF的所有组织病理学标准,但也呈现出海绵形成样模式的表皮黏蛋白沉积症。共15份标本进行了免疫组织化学研究,7份标本检测了T细胞受体(TCR)基因重排。纳入20份海绵状皮炎的活检标本作为对照。

结果

基于组织病理学标准和黏蛋白的胶体铁染色,我们在所有30例呈海绵形成样模式的MF病例中均证实了表皮黏蛋白沉积症的存在。15份标本的免疫组织化学显示泛T细胞抗原CD5(10/15)和CD7(14/15)显著缺失;在6例患者的7份标本中检测到TCR克隆性,支持MF的诊断。

结论

我们报告了有助于区分呈海绵形成样模式的伴有表皮黏蛋白沉积症的MF与海绵状皮炎的组织病理学标准。

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