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与肉芽肿性皮肤松弛症相关的皮肤T细胞淋巴瘤。

Cutaneous T-cell lymphoma associated with granulomatous slack skin.

作者信息

Mouly F, Baccard M, Cayuela J M, Janssen F, Verola O, Flageul B, Morel P

机构信息

Department of Dermatology, Saint Louis Hospital, Paris, France.

出版信息

Dermatology. 1996;192(3):288-90. doi: 10.1159/000246389.

Abstract

Granulomatous slack skin disease (GSS) is a rare disorder characterized by bulky cutaneous lesions and epithelioid and giant cell granulomas with destruction of the dermal elastic tissue. We detail the observation of a 29-year-old man with clinical and histological features of GSS. Pendulous skin tumors were associated with typical clinical and immunohistochemical aspects of mycosis fungoides and with clonal rearrangement of the V gamma T-cell receptor gene in lesional skin. This case report supports cutaneous T-cell lymphoma as a cause of GSS.

摘要

肉芽肿性皮肤松弛症(GSS)是一种罕见的疾病,其特征为巨大的皮肤损害以及上皮样和巨细胞肉芽肿,并伴有真皮弹性组织的破坏。我们详细报告了一名29岁男性患者,其具有GSS的临床和组织学特征。下垂的皮肤肿瘤与蕈样肉芽肿的典型临床和免疫组化表现相关,且病变皮肤中存在VγT细胞受体基因的克隆重排。本病例报告支持皮肤T细胞淋巴瘤是GSS的病因。

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