Cutaneous Features of Anti-MDA-5 Antibody-Positive Amyopathic Dermatomyositis in a Sudanese Patient

Abstract

Clinically amyopathic dermatomyositis (CADM) is a rare form of DM characterized by unique cutaneous and pulmonary features with no muscle involvement. A subset of patients with CADM has a specific antibody known as anti-melanoma differentiation-associated protein 5 (MDA5). The systemic associations of anti-MDA-5 CADM warrant an early recognition and management to prevent fetal sequelae. It is seen more commonly in white and Asian female individuals. The clinical features of anti-MDA5 antibody-positive CADM in other ethnic groups are not well reported. Here, we describe a case of CADM with identified autoantibodies against MDA5 in a Sudanese female patient presenting with characteristic cutaneous features in association with MDA5 autoantibodies: ulcerated Gottron’s papules, painful palmar papules, shawl sign, and heliotrope sign. No evidence of pulmonary or systemic involvement was identified. Treatment with prednisolone and mycophenolate mofetil was initiated. This case emphasizes the importance of keeping a high level of suspicion and to recognize the unique clinical feature of this type of DM aiding in early treatment and preventing fatal outcomes.

Treatment of myopathy and cutaneous ulcers in anti-MDA5-positive dermatomyositis with triple therapy

“…A larger study that looked at cutaneous manifestations in various subtypes of DM noted MMF to be associated with remission of skin lesions while no such association was noted with IVIG 25. Resolution of cutaneous ulcers were noted with the combination of MMF and prednisone in two case reports 26 27. However, progression of cutaneous ulcerations was noted on similar regimen in another patient warranting the addition of IVIG 28…”

A Review of MDA-5 Dermatomyositis and Associated Interstitial Lung Disease

“…MDA-5 DM presents with the classical cutaneous signs of DM, including Gottron's papules, shawl sign, and heliotrope rash (Figure 3) [14]. Additionally, MDA-5 DM manifests with an inverse Gottron's sign (Figures 4 and 5B) that appears as keratotic papules in the skin folds of the palms and fingers [15].…”