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一项关于 15 例色素减退性蕈样肉芽肿的临床病理特征研究。

A study of clinicopathologic profile of 15 cases of hypopigmented mycosis fungoides.

机构信息

Department of Dermatology, Seth GS Medical College and KEM Hospital, Parel, Mumbai-400 012, India.

出版信息

Indian J Dermatol Venereol Leprol. 2011 Mar-Apr;77(2):167-73. doi: 10.4103/0378-6323.77456.

DOI:10.4103/0378-6323.77456
PMID:21393946
Abstract

BACKGROUND

Mycosis fungoides (MF) is cutaneous lymphoma of the T-cell lineage. Hypopigmented MF is a clinical variant of MF, described mainly in Asians. This is a retrospective clinicopathologic analysis of hypopigmented MF at a tertiary care center.

AIMS

To describe the clinicopathologic profile of hypopigmented MF.

METHODS

Records of clinicopathologic notes over a 5-year period ranging from January 2005 up to December 2009 were reviewed over a period of 3 months, of which 15 cases were diagnosed with hypopigmented MF based on clinicopathologic correlation.

RESULTS

Hypopigmented MF was found to be more common in males, and between second and fourth decades of life. The latent period between onset and diagnosis was around 3.83 years. Most of the patients were asymptomatic 80% (12/15), with skin changes of subtle atrophy in 46.66% (7/15), scaling in 20% (3/15) and focal changes of poikiloderma in 26.66% (4/15) patients. Most common sites of distribution of the lesions were the trunk and extremities. Many of the cases had been clinically mistaken for Hansen's disease prior to correct diagnosis. Marked epidermotropism and tagging of epidermis by large lymphocytes characterizes the condition histopathologically. Of the 15 cases, immunohistochemistry was possible in 10 cases, of which 8 showed predominant CD8 positive epidermotropic infiltrates and two cases showed absence of CD8 positive and CD4 positive lymphocytic infiltrate in the epidermis.

CONCLUSION

Hypopigmented MF presents as hypopigmented asymptomatic patches without any erythema or infiltration in its early stage and mimics Hansen's disease. Skin biopsy clinches the diagnosis.

摘要

背景

蕈样肉芽肿(MF)是 T 细胞谱系的皮肤淋巴瘤。色素减退型 MF 是 MF 的一种临床变异型,主要见于亚洲人。这是一家三级保健中心回顾性临床病理分析色素减退型 MF 的结果。

目的

描述色素减退型 MF 的临床病理特征。

方法

对 2005 年 1 月至 2009 年 12 月期间为期 5 年的临床病理记录进行回顾性分析,在 3 个月的时间内,根据临床病理相关性诊断了 15 例色素减退型 MF。

结果

色素减退型 MF 多见于男性,发病年龄在 20 至 40 岁之间。发病到诊断的潜伏期约为 3.83 年。大多数患者无症状(80%,12/15),皮肤改变为轻微萎缩(46.66%,7/15)、鳞屑(20%,3/15)和斑驳性皮肤异色症(26.66%,4/15)。病变最常见的分布部位是躯干和四肢。许多病例在确诊前曾被误诊为汉森病。组织病理学表现为大淋巴细胞显著亲表皮性和表皮标签。在 15 例病例中,有 10 例进行了免疫组化检查,其中 8 例表现为主要的 CD8 阳性亲表皮性浸润,2 例表现为表皮内缺乏 CD8 阳性和 CD4 阳性淋巴细胞浸润。

结论

色素减退型 MF 在早期表现为色素减退的无症状斑块,无红斑或浸润,类似于汉森病。皮肤活检可确诊。

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