Dermatomyositis

This patient presented with a few week history of progressive weakness affecting the four limbs associated with muscle pain. He found it increasingly difficult to lift up his arms to above his shoulder level and also to climb stairs. There was in addition unintentional weight loss of 20 pounds (baseline of 180 pounds) during the past 2 months. Physical examination revealed the presence of a heliotropic skin rash with mild oedema especially prominent over the right eyelids and perioribital tissue. There was in addition presence of Gottron’s papules noted over the knuckles of the hands. Neurological examination revealed prominent bilateral, symmetrical, proximal muscle weakness over all four limbs with preserved distal limb power, normal reflexes and downgoing plantar responses. The patient had significant difficultly standing up from a sitting position and was only able to stand up with assistance by pressing against the handles of the chair. Muscle tenderness was also present. Sensory examination and cranial nerve examination was unremarkable. Subsequent investigations including blood tests for muscle enzymes and electromyography confirmed the presence of an active inflammatory myositis. Together with the skin changes, a diagnosis of dermatomyositis was made. A search for an underlying malignancy was made including blood tests for tumour markers as well as a whole body positron-emission tomography and computed tomography scan. The patient was subsequently diagnosed to have an underlying nasopharyngeal carcinoma resulting in dermatomyositis.