颈部和背部的非典型皮肤病是抗-HMGCR抗体阳性肌病的皮肤表现。

IF 2.9 4区 医学 Q3 IMMUNOLOGY BMC Immunology Pub Date : 2024-05-11 DOI:10.1186/s12865-024-00622-2
Takashi Kurashige, Rie Nakamura, Tomomi Murao, Naoko Mine, Mayu Sato, Riho Katsumata, Yuhei Kanaya, Yoriko Dodo, Tomohito Sugiura, Tomohiko Ohshita
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引用次数: 0

摘要

背景:免疫介导的坏死性肌病(IMNM)是一种特发性炎症性肌病(IIM)。尽管免疫介导的坏死性肌病(IMNM)患者被认为不会出现皮疹,但一些报告显示,抗-3-羟基-3-甲基戊二酰辅酶 A 还原酶(HMGCR)抗体阳性的免疫介导的坏死性肌病(HMGCR-IMNM)患者会出现非典型皮肤症状。HMGCR-IMNM患者皮肤病的发病率和表型尚不完全清楚:在2015年4月至2022年8月期间确诊的100例IIM患者中,有34例(34%)出现了某种形式的皮肤状况,其中27例有典型的皮疹;这包括13例皮肌炎(DM)患者、8例抗合成酶综合征(ASS)患者和6例IMNM患者。与此同时,19 名 HMGCR-IMNM 患者中有 8 人(42%)出现了非典型皮损,而其他 IIM 患者中没有人出现非典型皮损(P 结论):HMGCR-IMNM患者的颈部和背部经常出现非典型皮肤病。这些病变的皮肤活检标本显示出与肌肉活检标本相同的 Bcl-2 阳性淋巴细胞浸润,而不考虑不同的皮肤大体结果。因此,这种非典型皮肤病可能提示存在 HMGCR-IMNM。
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Atypical skin conditions of the neck and back as a dermal manifestation of anti-HMGCR antibody-positive myopathy.

Background: Immune-mediated necrotizing myopathy (IMNM) is an idiopathic inflammatory myopathy (IIM). Though patients with IMNM were not considered to show skin rash, several reports have showed atypical skin conditions in patients with anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) antibody-positive IMNM (HMGCR-IMNM). The incidence and phenotype of skin conditions in patients with HMGCR-IMNM are not fully known.

Results: Among the 100 IIM patients diagnosed from April 2015 through August 2022, 34 (34%) presented some form of skin condition, with 27 having typical skin rashes; this included 13 patients with dermatomyositis (DM), 8 with anti-synthetase syndrome (ASS), and 6 with IMNM. Meanwhile, 8 of 19 patients with HMGCR-IMNM (42%) presented atypical skin lesions, but no patients with other IIMs did (p < 0.001). Skin eruption with ash-like scales was observed in four HMGCR-IMNM patients, and non-scaly red patches and lumps in the other four patients; accordingly, their skin manifestations were considered as other dermal diseases except for IIM. However, skin and muscle biopsies revealed the atypical skin conditions of patients with HMGCR-IMNM to have the same pathological background, formed by Bcl-2-positive lymphocyte infiltrations.

Conclusions: HMGCR-IMNM patients frequently have atypical skin conditions of the neck and back. Skin biopsy specimens from these lesions showed the same Bcl-2-positive lymphocytic infiltrations as muscle biopsy specimens regardless of the different gross dermal findings. Thus, such atypical skin conditions may be suggestive for HMGCR-IMNM.

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来源期刊
BMC Immunology
BMC Immunology 医学-免疫学
CiteScore
5.50
自引率
0.00%
发文量
54
审稿时长
1 months
期刊介绍: BMC Immunology is an open access journal publishing original peer-reviewed research articles in molecular, cellular, tissue-level, organismal, functional, and developmental aspects of the immune system as well as clinical studies and animal models of human diseases.
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