Dermatomyositis Presenting With Focal Scleroderma-Like Skin Changes

Abstract

To describe an unusual focal scleroderma-like skin changes in patient with dermatomyositis. Review of clinical records, laboratory investigations, and muscle and skin biopsies. The patient developed unusual skin lesions characterized by symmetrical atrophy and hardening of focal skin and muscle over the lateral upper arms and posterior shoulders, and the left temporal and pectoral areas. Extensive blood work-up for scleroderma and other connective-tissue diseases was negative. A skin biopsy showed distinct pathologic features including increased interstitial mucin, hyperpigmentation, and perivascular lymphocytic inflammation without severe fibrosis. The scleroderma-like skin changes and muscle weakness improved with immunotherapy. Dermatomyositis can manifest with focal scleroderma-like skin changes clinically and reticular erythematous mucinosis-like changes pathologically, and these distinct skin changes represent a new variant of skin lesions of dermatomyositis.