Erythematous auricular papules in the fatal cases of anti-MDA5 antibody-positive interstitial lung disease

Abstract

Introduction/objectives The anti-melanoma differentiation-associated gene 5 (Anti-MDA5) antibody is associated with rapidly progressive interstitial lung disease (RP-ILD) and clinically amyopathic dermatomyositis (CADM). The predictors of treatment response would help in classifying the subgroups and decision-making. Here, we aimed to report an observational skin lesion that might be associated with a grave prognosis in six patients with anti-MDA5 antibody-positive ILD. Methods This case series included 6 anti-MDA5 antibody-positive ILD patients, who were admitted to Songklanagarind Hospital between January 2018 and June 2020. Their medical records were reviewed for clinical phenotypes, laboratory results, imaging studies, treatment, and outcomes. The cutaneous manifestations associated with fatal outcomes were observed and reported. Results Among 6 patients with anti-MDA5 antibody-positive ILD, 5 patients had CADM and one patient had no skin involvement. Four patients manifested as RP-ILD within a few months. Three deaths occurred despite highly intensive immunosuppressive treatment. All the patients in the dead group exhibited erythematous papules on their auricles and a presence of pulmonary consolidation at lower lung fields was additionally observed. Conclusion Erythematous auricular papules may be a hallmark of grave prognosis in anti-MDA5 positive CADM with ILD.

Dermatomyositis With Anti-MDA5 Antibodies: Bioclinical Features, Pathogenesis and Emerging Therapies

“…Less specific auricular skin lesions have also been described in DM with anti-MDA5 Abs, such as antihelix/helix violaceous macules and erythematous auricular papules ( 34 , 35 ). These particular cutaneous phenotypes, seldom mentioned in the literature, could be clinical markers of poor prognosis, although most previous studies examining auricular skin lesions in the disease have been limited to isolated case reports ( 36 ). Other less specific mucocutaneous lesions have been described in DM with anti-MDA5 Abs.…”

Cutaneous Manifestations and Their Corresponding Dermoscopic Features in Patients with Dermatomyositis

“…Dermoscopy enables better visualization of the three distinctive components, forming poikiloderma -cutaneous atrophy, telangiectasias, and macular pigmentary changes (Figure 3). Antihelix/helix violaceous macules, erythematous auricular papules and even small ulcerations of the ears have been described previously in association with anti -MDA 5 antibody phenotype and have been proposed as a prognostic marker for fatal pulmonary disease [21][22][23]. The latter findings might be explained by an underlying vasculopathy.…”

Violaceous Macules on the Auricles: A Clinical Sign of Dermatomyositis

“…Dermatomyositis can present with a myriad of cutaneous manifestations. Violaceous macules on the ears have recently been described in anti-melanoma differentiation-associated gene 5 (MDA-5) dermatomyositis and may correlate with a poor prognosis [ 1 ]. As the violaceous macules and ulcers are predominantly located in anatomic protuberances in the auricle, a pressure-induced microangiopathy has been proposed as the pathogenic mechanism [ 2 ].…”