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从局限性硬皮病(硬斑病)到皮下脂膜炎样T细胞淋巴瘤:病例报告

From circumscribed scleroderma (morphea) to subcutaneous panniculitis-like T-cell lymphoma: case report.

作者信息

Troskot Nina, Lugović Liborija, Situm Mirna, Vucić Majda

机构信息

Department of Dermatology and Venerology, Sestre milosrdnice University Hospital, Vinogradska c. 29, 10000 Zagreb, Croatia.

出版信息

Acta Dermatovenerol Croat. 2004;12(4):289-93.

PMID:15588564
Abstract

Subcutaneous panniculitis-like T-cell lymphoma is a rare form of non-Hodgkin lymphoma. It presents clinically as panniculitis, with erythematous, firm subcutaneous infiltrates and recurrent papulonodules. A 45-year-old male Croat presented with a 6-month history of tender erythematous subcutaneous skin lesions without systemic symptoms. Analysis of a lesion biopsy specimen showed circumscribed scleroderma (morphea). During hospitalization, skin biopsies from two subcutaneous lesions on the stomach were repeated, and pathohistologic analysis corresponded primarily to panniculitis in the context of morphea. Pathohistologic analysis and immunohistochemistry of a specimen obtained by tumor excision from the back indicated subcutaneous panniculitis-like T-cell lymphoma. Histologic analysis revealed a dense infiltrate of atypical T-lymphoid cells expressing CD8(+) phenotype located in the subcutaneous tissue with histiocyte-phagocytizing apoptotic cells. Specific hematologic analysis and medical treatment were continued. This case report illustrates the importance of continuous follow-up with repeat biopsy and use of immunohistologic techniques for early diagnosis.

摘要

皮下脂膜炎样T细胞淋巴瘤是一种罕见的非霍奇金淋巴瘤。临床上表现为脂膜炎,有红斑、坚实的皮下浸润及反复发作的丘疹结节。一名45岁的克罗地亚男性患者,有6个月的皮下皮肤压痛性红斑病变史,无全身症状。病变活检标本分析显示为局限性硬皮病(硬斑病)。住院期间,对胃部两个皮下病变再次进行皮肤活检,病理组织学分析主要符合硬斑病背景下的脂膜炎。对背部肿瘤切除标本进行的病理组织学分析和免疫组化显示为皮下脂膜炎样T细胞淋巴瘤。组织学分析显示皮下组织中有密集浸润的表达CD8(+)表型的非典型T淋巴细胞,伴有组织细胞吞噬凋亡细胞。继续进行了特定的血液学分析和医学治疗。本病例报告说明了持续随访、重复活检及使用免疫组化技术进行早期诊断的重要性。

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