Department of Pathology, Icahn School of Medicine at Mount Sinai, New York, New York, USA.
Dermatology Physician of Connecticut, Connecticut, USA.
J Cutan Pathol. 2024 Oct;51(10):799-806. doi: 10.1111/cup.14691. Epub 2024 Jul 15.
Despite the advancements in the categorization of clinically amyopathic dermatomyositis (CADM), the classification and diagnosis of its subtypes are still challenging. The aim of our study was to describe the clinicopathological features of CADM and assess the differences between amyopathic dermatomyositis (ADM) and hypomyopathic dermatomyositis (HDM).
This retrospective study included 43 patients with CADM diagnosed at our institution from 2016 to 2020. Patients were subclassed into ADM (n = 30) and HDM (n = 13) groups to assess their clinicopathological differences.
All included patients had characteristic cutaneous manifestations of dermatomyositis; 67.4% had myositis-associated auto-antibodies, including ANA (32.6%), RNP (14.0%), anti-Ro52 (9.3%), anti-p155/140 (7.0%), rheumatoid factor (7.0%), anti-NXP-2 (4.7%), anti-MDA5 (2.3%), and anti-Jo-1 (2.3%) antibodies. One patient had associated interstitial lung disease, and another patient had oral squamous cell carcinoma. The histopathological findings included mucin deposition (69.8%), telangiectasia (65.1%), lymphocytic infiltrate (48.8%), vacuolar interface dermatitis (46.5%), and epidermal atrophy (14.0%). Compared to patients with HDM, ADM patients were significantly less likely to have epidermal atrophy, 3.3% versus 38.5% (p = 0.006), and more likely to have mucin deposition, 80.0% versus 46.2% (p = 0.028).
We described the clinicopathological features of CADM and highlighted the distinctions between ADM and HDM dermatopathologic findings. This information may prove helpful in diagnosing ambiguous lesions.
尽管在临床无肌病性皮肌炎（CADM）的分类方面取得了进展，但该病亚型的分类和诊断仍然具有挑战性。我们的研究旨在描述 CADM 的临床病理特征，并评估无肌病性皮肌炎（ADM）和低肌病性皮肌炎（HDM）之间的差异。
本回顾性研究纳入了 2016 年至 2020 年期间在我院诊断为 CADM 的 43 例患者。将患者分为 ADM（n=30）和 HDM（n=13）两组，以评估其临床病理差异。
所有纳入患者均有皮肌炎的特征性皮肤表现；67.4%的患者有肌炎相关自身抗体，包括抗核抗体（ANA）（32.6%）、核糖核蛋白（RNP）（14.0%）、抗 Ro52 抗体（9.3%）、抗 p155/140 抗体（7.0%）、类风湿因子（7.0%）、抗 NXP-2 抗体（4.7%）、抗 MDA5 抗体（2.3%）和抗 Jo-1 抗体（2.3%）。1 例患者合并间质性肺病，另 1 例患者合并口腔鳞状细胞癌。组织病理学表现包括黏蛋白沉积（69.8%）、毛细血管扩张（65.1%）、淋巴细胞浸润（48.8%）、空泡性界面皮炎（46.5%）和表皮萎缩（14.0%）。与 HDM 患者相比，ADM 患者表皮萎缩的发生率明显较低（3.3%对 38.5%，p=0.006），而黏蛋白沉积的发生率明显较高（80.0%对 46.2%，p=0.028）。
我们描述了 CADM 的临床病理特征，并强调了 ADM 和 HDM 皮肤病理表现之间的区别。这些信息可能有助于诊断有疑问的病变。
