Amyopathic dermatomyositis or dermatomyositis-like skin disease: retrospective review of 16 cases with amyopathic dermatomyositis

Abstract

Amyopathic dermatomyositis (ADM) is characterized by the presence of dermatomyositis (DM) for 6 months or more in individuals who have normal muscle enzymes and no clinically significant muscle weakness. The aim of the study was to investigate the initial laboratory data, clinical manifestations, complications, and clinical outcomes of patients with the diagnosis of ADM. We reported 16 cases with the cutaneous findings of dermatomyositis without clinical or laboratory evidence of muscle disease for at least 2 years after onset of the skin manifestations in the Department of Dermatology and Rheumatology at Shanghai Ruijin Hospital between 1998 and 2004. All patients had Gottron's papules, periungual erythema/telangiectasia, and violaceous discoloration of the face, neck, upper chest, and back at some time during the course of their disease. Follow-up of 1 to 10 years after diagnosis found muscle weakness in three patients (18.75%) within 5 years of diagnosis. One patient (6.15%) was rediagnosed as chronic cutaneous lupus erythematosus (CCLE). Four patients (25%) had associated malignancies. Twelve patients (75%) had radiographic evidence indicative of interstitial fibrosis irrespective of respiratory symptoms. Patients with ADM appear to be at risk for developing the same potentially fatal disease complications as those patients with DM (e.g., interstitial lung disease and internal malignancy). These cases further emphasize that the cutaneous manifestations of dermatomyositis are pathognomonic for DM and we propose the term dermatomyositis-like skin disease as a better designation than amyopathic dermatomyositis to describe this distinctive subset of cutaneous symptoms. Dermatomyositis-like skin disease is a complex syndrome, which includes the characteristic cutaneous eruption of dermatomyositis without clinical evidence of muscle disease. Our findings suggest that patients diagnosed with this syndrome are at risk for fatal interstitial lung disease, malignancy, and/or delayed onset of DM or CCLE. Cautious systematic clinical trials should be considered for this group of patients.

Response of Mucocutaneous Lesions to Rituximab in a Case of Melanoma Differentiation Antigen 5-Related Dermatomyositis

“…Corticosteroids such as prednisone or (methyl)prednisolone are generally used as first-line drugs, with more or less satisfying responses depending on the studies. Hydroxychloroquine has been tried against DM/CADM skin lesions with responses that seem to be less satisfying than in other autoimmune conditions [21,22]. The use of thalidomide, high-dose immunoglobulins or immunosuppressive agents such as MMF, azathioprine, methotrexate, cyclophosphamide, cyclosporine A, oral tacrolimus, or biological agents such as etanercept or infliximab have also been reported with various success.…”

Clinical manifestations of dermatomyositis and clinically amyopathic dermatomyositis patients with positive expression of anti–melanoma differentiation–associated gene 5 antibody

“…Clinical manifestations of this disease spectrum are diverse, with various degrees of muscle, skin, and lung involvement or malignant diseases. We tend to use the term "dermatomyositis-like skin syndrome" to describe those patients who have distinctive or predominantly cutaneous manifestations of DM without clinical evidence of myositis, since patients diagnosed with this syndrome are at a higher risk for developing interstitial lung disease (ILD), malignancy, and/or delayed onset of myositis (2). Patients with DM/CADM showed a lower 5-year survival rate than patients with PM (3)(4)(5).…”

Gottron Papules and Gottron Sign with Ulceration: A Distinctive Cutaneous Feature in a Subset of Patients with Classic Dermatomyositis and Clinically Amyopathic Dermatomyositis

“…Among these, the exclusively cutaneous or predominantly cutaneous form of DM or CADM showed the lowest survival rates 2,3,4,5 .…”