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黏液性水肿性苔藓伴意义未明的单克隆丙种球蛋白病:一例报告及文献复习

Lichen myxedematosus associated with monoclonal gammopathy of undetermined significance: A case report and literature review.

作者信息

Huang Hua, Qian Shen-Xian

机构信息

Department of Hematology, Affiliated Hangzhou First People's Hospital, Zhejiang University School of Medicine, Hangzhou, China.

出版信息

Front Med (Lausanne). 2023 Mar 8;10:1118555. doi: 10.3389/fmed.2023.1118555. eCollection 2023.

DOI:10.3389/fmed.2023.1118555
PMID:36968834
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10032400/
Abstract

Lichen myxedematosus (LM) is an idiopathic cutaneous mucinosis disorder, and monoclonal gammopathy of undetermined significance (MGUS) is a preneoplastic plasma cell disease with a monoclonal increase in globulin. Patients with LM combined with monoclonal gammopathy are normally diagnosed with scleromyxedema. However, we report a case of generalized papules combined with MGUS in a 78-year-old man who was eventually diagnosed with atypical or intermediate forms of LM because it only involved the skin, and the pathological type was not consistent with scleromyxedema. Few cases of atypical or intermediate forms of LM have been reported, so the course of atypical or intermediate forms of LM is unpredictable. We report the diagnosis and treatment of a case of atypical forms of LM to discuss the current understanding of the disease, hoping to provide a reference for clinical research on this disease.

摘要

黏液水肿性苔藓(LM)是一种特发性皮肤黏蛋白沉积症,意义未明的单克隆丙种球蛋白病(MGUS)是一种肿瘤前期浆细胞疾病,球蛋白呈单克隆性增加。LM合并单克隆丙种球蛋白病的患者通常被诊断为硬化性黏液水肿。然而,我们报告了一例78岁男性患者,其患有泛发性丘疹并合并MGUS,最终被诊断为非典型或中间型LM,因为该病例仅累及皮肤,且病理类型与硬化性黏液水肿不一致。非典型或中间型LM的病例报道较少,因此其病程难以预测。我们报告了一例非典型LM的诊断和治疗情况,以探讨目前对该疾病的认识,希望为该病的临床研究提供参考。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8b1e/10032400/4c6f9bbc21f5/fmed-10-1118555-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8b1e/10032400/4fae320f2169/fmed-10-1118555-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8b1e/10032400/b29b708f9bd0/fmed-10-1118555-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8b1e/10032400/db68a2092d4d/fmed-10-1118555-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8b1e/10032400/4c6f9bbc21f5/fmed-10-1118555-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8b1e/10032400/4fae320f2169/fmed-10-1118555-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8b1e/10032400/b29b708f9bd0/fmed-10-1118555-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8b1e/10032400/db68a2092d4d/fmed-10-1118555-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8b1e/10032400/4c6f9bbc21f5/fmed-10-1118555-g004.jpg

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本文引用的文献

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衰老过程中的基因组不稳定性和遗传异质性:来自克隆性造血(CHIP)、单克隆丙种球蛋白病(MGUS)和单克隆B淋巴细胞增多症(MBL)的见解。
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