Dermatomyositis - Presentation

Presentation

Dermatomyositis occurs more commonly in female patients. It presents as a proximal symmetrical muscle weakness with vasculitis affecting the skin, muscles and internal organs. Patients find it hard to raise their arms to comb their hair or walk up the stairs due to the proximal muscle weakness. It can be severe enough to affect the muscles needed for speech and swallowing and is also known to cause respiratory compromise. Calcinosis can occur in the skin, joints and tissues. Upon investigation, characteristically one would find an elevated ESR and CPK along with typical EMG findings of spontaneous muscle fibrillation and short polyphasic muscle potentials.

Gottron lesions, scaly erythematous eruptions or red patches overlying the knuckles, elbows, and knees are a characteristic feature of Dermatomyositis. Other skin manifestations involve periungual telangiectasias and a heliotropic (purple) rash over the upper eyelids.

X-ray findings sometimes include dystrophic calcifications in the muscles, and patients may or may not notice small calcium deposits under the skin. Many do not have any calcium deposits of any kind. The rash also may come and go, and may not be dependent on the severity of the muscle involvement at the time. "Gottron's papules" (pink patches on the knuckles and other extensor surfaces) and priapism are associated with this disorder.