Juvenile dermatomyositis with bilateral progressive knee flexion contracture.

Overview

Juvenile dermatomyositis is a multisystem, inflammatory vasculopathy that primarily affects muscles and skin. Calcinosis is one of the most debilitating complications affecting patients with juvenile dermatomyositis. Calcifications resulting from calcinosis frequently are located on the elbows, knees, and other joints and can cause considerable disability with severe pain, joint contractures, skin ulcers, and muscle atrophy. Many therapies for calcinosis have been reported including diltiazem, probenecid, and alendronate. We report a patient surgically treated for bilateral knee flexion contractures with the Ilizarov technique. At 2.5 years' followup, the patient had full extension of both knees with 0 degree to 50 degrees flexion and was walking independently. The Ilizarov technique provides an important option for correcting knee flexion contractures secondary to calcinosis in juvenile dermatomyositis.