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Introduction: Systemic lupus erythematosus (SLE) is an autoimmune disorder involving multiple organs, predominantly seen in women of child-bearing age. Erythroderma is described in patients with subacute cutaneous lupus erythematosus (SCLE) but is rare in SLE. Among the cardiac manifestations, pericarditis is common but myocarditis is rare. We report a case of severe SLE presenting with erythroderma, pancytopenia, arthralgia and myocarditis. Case Report: A 67-year-old male with a background history of hypertension, transient ischemic attack and polymyalgia rheumatica presented with severe erythroderma, malaise, arthralgia, weight loss and was found to be pancytopenic. His antinuclear antibody (ANA) was positive and double stranded DNA (dsDNA) was more than 200 IU/mL with very low complement levels. He developed lupus associated myocarditis with moderately impaired global left ventricular systolic function. He was initially started on steroids and hydroxychloroquine. But as he developed steroid induced myopathy, it was gradually tapered off and mycophenolate mofetil was started. He responded well to the treatment. He satisfied 8 of the 17 systemic lupus international collaborating clinics (SLICC) criteria establishing a diagnosis of systemic lupus erythematosus (SLE). His systemic lupus erythematosus disease activity index (SLEDAI) score came down from 17 to 2 over a period of six months of follow-up. Conclusion: Systemic lupus erythematosus may present with erythroderma. Careful clinical examination is important in all potentially multi-system diseases.

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