Dermatology Service, Hospital del Sureste, Arganda del Rey, Madrid, Spain.
Dermatology Service, Hospital Universitario La Princesa, Madrid, Spain.
J Dtsch Dermatol Ges. 2023 Jul;21(7):741-748. doi: 10.1111/ddg.15089. Epub 2023 May 23.
Melanoma differentiation-associated gene 5 antibody (anti-MDA5) in dermatomyositis (DM) is associated with rapidly progressive interstitial lung disease and poor prognosis. Early diagnosis is key to improving the prognosis of these patients. The aim was to confirm cutaneous characteristics in patients with anti-MDA5 dermatomyositis and to explore new diagnostic markers for the presence of anti-MDA5 (anti-MDA5 ).
A multicenter cross-sectional retrospective cohort study of 124 patients diagnosed with DM, of which 37 were anti-MDA5 . Demographic data, laboratory data, and clinical manifestations were collected.
Anti-MDA5 DM is characterized by a distinct mucocutaneous phenotype that includes oral lesions, alopecia, mechanic's hands, palmar and dorsal papules, palmar erythema, vasculopathy, and skin ulceration. We found vasculopathy and digit tip involvement very frequently in anti-MDA5 patients (p <0.001), being a diagnostic marker of anti-MDA5 (OR, 12.355; 95% CI 2.850-79.263; p = 0.012 and OR, 7.447; 95% CI 2.103-46.718; p = 0.004, respectively). The presence of ulcers deserves special mention, especially in anti-MDA5 patients, because in our cohort, up to 97% of the anti-MDA5 patients had ulcers.
In patients with suspected DM with digit tip involvement or vasculopathy, the presence of anti-MDA5 antibodies must be ruled out, as it may be a clinical predictor.
在皮肌炎(DM)中,黑色素瘤分化相关基因 5 抗体(抗-MDA5)与快速进展性间质性肺病和不良预后相关。早期诊断是改善这些患者预后的关键。本研究旨在确认抗-MDA5 皮肌炎患者的皮肤特征,并探索抗-MDA5(抗-MDA5)存在的新诊断标志物。
这是一项多中心回顾性队列研究,共纳入 124 例诊断为 DM 的患者,其中 37 例为抗-MDA5。收集了患者的人口统计学数据、实验室数据和临床表现。
抗-MDA5 DM 的特征是独特的黏膜皮肤表型,包括口腔病变、脱发、机械性手、手掌和背部丘疹、手掌红斑、血管病变和皮肤溃疡。我们发现血管病变和指尖受累在抗-MDA5 患者中非常常见(p<0.001),是抗-MDA5 的诊断标志物(OR,12.355;95%CI 2.850-79.263;p=0.012 和 OR,7.447;95%CI 2.103-46.718;p=0.004)。值得特别提及的是溃疡的存在,尤其是在抗-MDA5 患者中,因为在我们的队列中,高达 97%的抗-MDA5 患者有溃疡。
在怀疑患有 DM 且指尖受累或血管病变的患者中,必须排除抗-MDA5 抗体的存在,因为它可能是一种临床预测因子。