P.14.3 Dermatomyositis in a patient with Behcet’s disease

Abstract

A case of dermatomyositis associated with Behcet's disease (BD) is described. A 51-year-old male with a 12-year history of BD, was currently on remission with colchicine therapy (0.5mg/day), presented with acute onset difficulty in walking and skin lesions. His neurological examination revealed proximal muscle weakness in both the upper and lower extremities. No myalgia was noted. He had cutaneous lesions typical for dermatomyositis on his face, neck, upper trunk and hands. Laboratory tests demonstrated normal findings except elevated ESR and serum CK level (639U/L). Muscle MRI of lower extremities revealed multiple patchy hyperintense lesions in pelvic and thigh muscles on STIR images. There was no muscle atrophy or fat replacement and it was commented as muscle inflammation. The clinical and laboratory findings were compatible with the diagnosis of dermatomyositis. He responded well to IV methylprednisolone within days. He continued on prednisolone therapy orally with gradual tapering. This case of Behcet's disease associated with dermatomyositis (DM) is a very rare one. Muscular involvement in BD is rarely described but DM in association with BD has been never reported. The underlying cause of BD and the precise pathogenic mechanisms responsible for DM are unknown. The association of BD and DM may provide insights into genetic predisposition and pathogenesis of these complex and multisystemic autoimmune disorders.