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印度色素减退性蕈样肉芽肿的临床病理特征

Clinicopathologic profile of hypopigmented mycosis fungoides in India.

作者信息

Koorse Sharon, Tirumalae Rajalakshmi, Yeliur Inchara K, Jayaseelan Elizabeth

机构信息

Department of Pathology, St John 's Medical College and Hospital, Bangalore, India.

出版信息

Am J Dermatopathol. 2012 Apr;34(2):161-4. doi: 10.1097/DAD.0b013e31822e6877.

DOI:10.1097/DAD.0b013e31822e6877
PMID:22240772
Abstract

BACKGROUND

Hypopigmented mycosis fungoides (HMF) is an under recognized disease in India, which is often mistaken for Hansen disease or vitiligo, resulting in delayed diagnosis and treatment.

AIM

To describe the clinical, histopathologic and immunohistochemical features of HMF in Indian patients.

MATERIALS AND METHODS

All cases presenting as hypopigmented lesions that were signed out as MF between 2001 and 2009 (15 cases) were included. Clinical data and histopathology slides were reviewed. Immunostains for CD4, CD8, and CD1a were done, where tissue was available.

RESULTS

The age ranged from 14 to 38 years with a male preponderance. The commonest presentation was multiple hypopigmented patches on limbs and trunk with the duration of the lesions varying from 4 months to 14 years. All cases showed a psoriasiform/lichenoid epidermal pattern, disproportionate epidermotropism, basilar tagging of lymphocytes, monomorphous lymphocytes, haloed lymphocytes, and wiry dermal collagen. Other important findings were infiltration of hair follicles, larger epidermal lymphocytes, atypia of dermal lymphocytes, and stuffed dermal papillae. Dermal edema was absent in all cases. Immunohistochemistry done on 10 cases showed a CD8 phenotype in 6 cases and CD4 phenotype in the remaining 4 cases.

CONCLUSIONS

Histopathology supplemented by immunohistochemistry is reliable in making a diagnosis of HMF. It is important to be aware of this uncommon, yet significant disease.

摘要

背景

色素减退性蕈样肉芽肿(HMF)在印度是一种未被充分认识的疾病,常被误诊为麻风病或白癜风,导致诊断和治疗延迟。

目的

描述印度患者HMF的临床、组织病理学和免疫组化特征。

材料与方法

纳入2001年至2009年间所有表现为色素减退性皮损且病理诊断为MF的病例(15例)。回顾临床资料和组织病理学切片。对有组织的病例进行CD4、CD8和CD1a免疫染色。

结果

年龄范围为14至38岁,男性居多。最常见的表现是四肢和躯干出现多个色素减退斑,皮损持续时间从4个月至14年不等。所有病例均表现为银屑病样/苔藓样表皮模式、不成比例的亲表皮现象、淋巴细胞基底附着、单一形态淋巴细胞、晕状淋巴细胞和致密的真皮胶原纤维。其他重要发现包括毛囊浸润、较大的表皮淋巴细胞、真皮淋巴细胞异型性和真皮乳头填充。所有病例均无真皮水肿。对10例病例进行的免疫组化显示,6例为CD8表型,其余4例为CD4表型。

结论

组织病理学辅以免疫组化对HMF的诊断可靠。认识到这种罕见但重要的疾病很重要。

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