Mouly F, Baccard M, Cayuela J M, Janssen F, Verola O, Flageul B, Morel P
Department of Dermatology, Saint Louis Hospital, Paris, France.
Dermatology. 1996;192(3):288-90. doi: 10.1159/000246389.
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.
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。
