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伴有副蛋白血症的坏死性黄色肉芽肿的组织病理学

Histopathology of necrobiotic xanthogranuloma with paraproteinemia.

作者信息

Finan M C, Winkelmann R K

出版信息

J Cutan Pathol. 1987 Apr;14(2):92-9. doi: 10.1111/j.1600-0560.1987.tb00479.x.

DOI:10.1111/j.1600-0560.1987.tb00479.x
PMID:3597917
Abstract

Fifty-three cutaneous biopsies from 22 patients with necrobiotic xanthogranuloma (NXG) were reviewed. One or more biopsies from each patient displayed a typical pattern of palisading histiocytic xanthogranuloma with bands of hyaline necrobiosis. Multiple foam cells and Touton giant cells were present, and atypical, bizarre-appearing foreign body giant cells were characteristic. Cholesterol clefts were found in 18 specimens, and lymphoid nodules were found in 24, 8 of which had germinal center formation. Twenty-one specimens contained foci of plasma cells, and these were located perivascularly, at the periphery of lymphoid nodules, and, as infiltrates, between dermal collagen bundles. Unique features were xanthogranulomatous panniculitis, often appearing as Touton cell panniculitis, and a rare but distinctive palisading cholesterol cleft granuloma. The cytoplasm of giant cells and histiocytes contained PAS-positive, diastase-resistant polysaccharide. Examination of 3 cases by electron microscopy revealed dendritic cells in the epidermis and vacuolated dermal histiocytes. S-100 antibody was negative in 3 cases. Leukocyte monoclonal antibody studies in 6 patients showed predominantly T-helper lymphocytes within the granuloma. NXG is a T-helper cell, non-X histiocytic granuloma with a distinctive histopathology.

摘要

对22例坏死性黄色肉芽肿(NXG)患者的53份皮肤活检标本进行了回顾性研究。每位患者的一份或多份活检标本显示出具有透明坏死带的栅栏状组织细胞性黄色肉芽肿的典型模式。存在多个泡沫细胞和杜顿巨细胞,非典型的、形态怪异的异物巨细胞是其特征。18份标本中发现了胆固醇裂隙,24份标本中发现了淋巴小结,其中8份有生发中心形成。21份标本含有浆细胞灶,这些浆细胞位于血管周围、淋巴小结周边以及真皮胶原束之间的浸润部位。独特的特征包括黄色肉芽肿性脂膜炎,常表现为杜顿细胞脂膜炎,以及一种罕见但独特的栅栏状胆固醇裂隙肉芽肿。巨细胞和组织细胞的细胞质含有PAS阳性、抗淀粉酶的多糖。对3例进行电子显微镜检查发现表皮中有树突状细胞和真皮组织细胞空泡化。3例S-100抗体呈阴性。对6例患者进行白细胞单克隆抗体研究显示肉芽肿内主要为辅助性T淋巴细胞。NXG是一种辅助性T细胞、非X组织细胞性肉芽肿,具有独特的组织病理学表现。

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