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

• Main Authors: Nouf Alqahtani, Majed Aleissa
• Format: Article
• Language: English
• Published: Karger Publishers 2021-09-01
• Series: Case Reports in Dermatology
• Subjects: dermatomyositis; amyopathic; clinically amyopathic dermatomyositis; melanoma differentiation-associated protein 5 antibody
• Online Access: https://www.karger.com/Article/FullText/519104

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.