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多中心性网状组织细胞增生症

Multicentric reticulohistiocytosis.

作者信息

Tajirian Ani L, Malik Mohsin K, Robinson-Bostom Leslie, Lally Edward V

机构信息

Department of Dermatology, Brown Medical School, Rhode Island Hospital, Providence, RI 02903, USA.

出版信息

Clin Dermatol. 2006 Nov-Dec;24(6):486-92. doi: 10.1016/j.clindermatol.2006.07.010.

Abstract

Multicentric reticulohistiocytosis is a rare granulomatous disease of unknown etiology, characterized by cutaneous nodules and destructive arthritis. Skin lesions can cause significant deformity, and approximately half of affected patients develop a severe disabling arthritis. The disease is often associated with malignancy; however, the paraneoplastic nature of multicentric reticulohistiocytosis is not established. The diagnosis is confirmed by the presence of oncocytic ("ground-glass") histiocytes and multinucleated giant cells on histopathology of the cutaneous nodules and the synovial membrane.

摘要

多中心网状组织细胞增多症是一种病因不明的罕见肉芽肿性疾病,其特征为皮肤结节和破坏性关节炎。皮肤病变可导致严重畸形,约半数受累患者会发展为严重致残性关节炎。该疾病常与恶性肿瘤相关;然而,多中心网状组织细胞增多症的副肿瘤性质尚未明确。通过皮肤结节和滑膜组织病理学检查发现嗜酸性(“毛玻璃样”)组织细胞和多核巨细胞可确诊。

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