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用2-氯脱氧腺苷治疗坏死性黄色肉芽肿。

Treatment of necrobiotic xanthogranuloma with 2-chlorodeoxyadenosine.

作者信息

Sutton Leigh, Sutton Stephanie, Sutton Margaret

机构信息

University of Nebraska Medical Center, 2212 South 64th Plaza #401, Omaha, NE 68106, USA.

出版信息

Skinmed. 2013 Mar-Apr;11(2):121-3.

PMID:23745232
Abstract

A 71-year-old man presented with ulcerating yellow-red plaques of the back, chest, face, and extremities (Figure 1). He had a 10-year history of these lesions. The initial plaque appeared on the back followed by rapid progression of multiple similar lesions involving the face, chest, abdomen, and extremities. The plaques on his face were predominately in the periorbital and eyelid regions. Skin biopsy revealed an ulcerated epidermis. Dense sheets of foamy histiocytes were present in the dermis and contained foci of necrobiosis. Numerous Touton giant cells were present and cholesterol clefts were prominent. Special stains for organisms were negative. On further laboratory evaluation, serum protein electrophoresis revealed a monoclonal spike of 0.5 g/dL IgG lambda. Results from bone marrow biopsy were negative. Other significant laboratory findings included an elevated sedimentation rate and low serum complement. Clinical findings, histopathology, and laboratory evaluations were consistent with the diagnosis of necrobiotic xanthogranuloma (NXG).

摘要

一名71岁男性患者,背部、胸部、面部及四肢出现溃疡性黄红色斑块(图1)。这些皮损已有10年病史。最初的斑块出现在背部,随后多个类似皮损迅速进展,累及面部、胸部、腹部及四肢。其面部斑块主要位于眶周和眼睑区域。皮肤活检显示表皮溃疡。真皮层可见密集的泡沫状组织细胞片,并含有渐进性坏死灶。可见大量 Touton 巨细胞,胆固醇裂隙明显。特殊病原体染色为阴性。进一步实验室检查,血清蛋白电泳显示IgG λ单克隆峰为0.5 g/dL。骨髓活检结果为阴性。其他重要实验室检查结果包括血沉升高和血清补体降低。临床症状、组织病理学及实验室检查结果均符合渐进性坏死性黄色肉芽肿(NXG)的诊断。

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引用本文的文献

1
Systemic therapy of necrobiotic xanthogranuloma: a systematic review.坏死性黄色肉芽肿的系统性治疗:系统评价。
Orphanet J Rare Dis. 2022 Mar 24;17(1):132. doi: 10.1186/s13023-022-02291-z.
2
A Multicenter Cross-Sectional Study and Systematic Review of Necrobiotic Xanthogranuloma With Proposed Diagnostic Criteria.坏死性黄色肉芽肿的多中心横断面研究和系统评价及诊断标准的建议。
JAMA Dermatol. 2020 Mar 1;156(3):270-279. doi: 10.1001/jamadermatol.2019.4221.