Primary Systemic Amyloidosis Characterized by Multiple Keratinous Cysts: A Case Report.

IF 1.6 4区 医学 Q3 DERMATOLOGY
Xiaoou Lu, Weiwei Fu, Demin Xie, Fengjuan Li, Jiali Zhang, Da Wang, Yu Sun, Hongquan Chen
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引用次数: 0

Abstract

A 67-year-old male presented with plaques around the orbit and ecchymosis on the neck and back of hands for 2 years. Physical examination showed seborrheic-keratosis-like plaques around the orbit, ecchymosis on the neck and back of hands, as well as nail dystrophy. Serum λ light chain was positive. Histopathological examination demonstrated multiple keratinous cysts in the dermis and eosinophilic homogeneous material in the dermis, which was positive for Congo red with apple-green birefringence on polarized light. Kidney biopsy showed deposition of acellular eosinophilic material in the arteriole wall. Congo red staining was positive. A bone marrow biopsy revealed multiple myeloma. The diagnosis of primary systemic amyloidosis with multiple myeloma was made.

以多发角化囊肿为特征的原发性系统性淀粉样变性1例报告。
67岁男性,眼眶周围有斑块,颈部及手背有淤斑2年。体格检查显示眼眶周围有脂溢性角化样斑块,颈部和手背有瘀斑,指甲营养不良。血清λ轻链阳性。组织病理学检查示真皮多发角质囊肿,真皮内嗜酸性均质物质,偏光刚果红阳性,苹果绿双折射。肾活检显示小动脉壁有脱细胞性嗜酸性物质沉积。刚果红染色阳性。骨髓活检显示多发性骨髓瘤。诊断为原发性系统性淀粉样变合并多发性骨髓瘤。
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来源期刊
CiteScore
3.20
自引率
5.90%
发文量
174
审稿时长
3-8 weeks
期刊介绍: Journal of Cutaneous Pathology publishes manuscripts broadly relevant to diseases of the skin and mucosae, with the aims of advancing scientific knowledge regarding dermatopathology and enhancing the communication between clinical practitioners and research scientists. Original scientific manuscripts on diagnostic and experimental cutaneous pathology are especially desirable. Timely, pertinent review articles also will be given high priority. Manuscripts based on light, fluorescence, and electron microscopy, histochemistry, immunology, molecular biology, and genetics, as well as allied sciences, are all welcome, provided their principal focus is on cutaneous pathology. Publication time will be kept as short as possible, ensuring that articles will be quickly available to all interested in this speciality.
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