An elusive case of dermatomyositis

Dermatomyositis is an inflammatory myopathy of unknown aetiology. Muscle involvement may eventuate later in the disease course in some patients, who may present with typical skin disease without clinical signs of myopathy and are referred to as dermatomyositis sine myositis. A 48 year old female presented with intermittent urticaria like rashes, diffuse asymmetrical swelling of proximal limbs, pain in small joints of hands and fatiguability. Initial laboratory work-up for immune markers was negative. Three years later, she developed heliotrope rash and periorbital oedema with no evidence of muscle weakness and was labeled as amyopathic dermatomyositis. After an interval of one year, she developed profound weakness and significantly raised CPK. Patient responded well to steroids and Azathioprine and improved both clinically and biochemically.