Anti-melanoma differentiation-associated gene 5 (MDA5) dermatomyositis: a concise review with an emphasis on distinctive clinical features

Publication date: Available online 9 December 2017
Source:Journal of the American Academy of Dermatology
Author(s): Drew JB. Kurtzman, Ruth Ann Vleugels
Melanoma differentiation-associated gene 5 (MDA5) is a recently described autoantigen target in a subset of patients with dermatomyositis (DM). Anti-MDA5 DM is characterized by a unique mucocutaneous and systemic phenotype that includes cutaneous and oral ulceration, painful palmar papules, alopecia, panniculitis, arthritis, a lower incidence of myositis, and, importantly, an elevated risk of interstitial lung disease, with a potentially fatal course. Because the clinical features may differ substantially from those typically observed in cutaneous DM, the diagnosis is often overlooked, which may negatively affect patient outcomes. This review aims to familiarize the clinician with the distinctive clinical features of anti-MDA5 DM in order to enhance its recognition and to facilitate an appropriate screening and management strategy.

Teaser

Anti-MDA5 dermatomyositis exhibits unique clinical features including mucocutaneous ulceration, palmar papules, non-scarring alopecia, panniculitis, arthritis, and interstitial lung disease.Early intervention may reduce disease-related morbidity and improve survival in affected individuals.Knowledge of the salient features of anti-MDA5 dermatomyositis enhances its recognition and facilitates treatment.