Department of Neurology, NHO Kure Medical Center, Chugoku Cancer Center, Kure, Japan.
Department of Dermatology, NHO Kure Medical Center, Chugoku Cancer Center, Kure, Japan.
BMC Immunol. 2024 May 11;25(1):30. doi: 10.1186/s12865-024-00622-2.
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.
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.
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.
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 IMNM 患者不被认为会出现皮疹，但几项报告显示，抗 3-羟基-3-甲基戊二酰基辅酶 A 还原酶（HMGCR）抗体阳性的 IMNM（HMGCR-IMNM）患者存在非典型皮肤状况。HMGCR-IMNM 患者皮肤状况的发生率和表型尚未完全清楚。
在 2015 年 4 月至 2022 年 8 月期间诊断的 100 例 IIM 患者中，有 34 例（34%）存在某种形式的皮肤状况，其中 27 例有典型皮疹；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。同时，19 例 HMGCR-IMNM 患者中有 8 例（42%）出现非典型皮肤病变，但其他 IIM 患者均未出现（p<0.001）。4 例 HMGCR-IMNM 患者出现伴有灰烬样鳞屑的皮疹，4 例患者出现无鳞屑的红色斑块和肿块；因此，除了 IIM 外，他们的皮肤表现被认为是其他皮肤疾病。然而，皮肤和肌肉活检显示 HMGCR-IMNM 患者的非典型皮肤状况具有相同的病理背景，由 Bcl-2 阳性淋巴细胞浸润形成。
HMGCR-IMNM 患者常有颈部和背部的非典型皮肤状况。这些病变的皮肤活检标本显示与肌肉活检标本相同的 Bcl-2 阳性淋巴细胞浸润，而不管真皮的大体发现如何。因此，这些非典型皮肤状况可能提示 HMGCR-IMNM。
