Combination Therapy for Extensive Tumoral Calcinosis in Juvenile Dermatomyositis

A 8-year-old girl presented with pain in bilateral hip and thigh with progressive difficulty in getting up and walking for last 2 years. Patient had difficulty with activities such as rising from a chair, climbing stairs, lifting objects, or washing her hair. There was no dysphagia, dysphonia, difficulty in swallowing and no weakness in the muscles of respiration. On examination child was very thin with areas of scalp alopecia. Multiple small, flat-topped, reddish-purple, non-tender papules were seen on the back of the hands and fingers indicative of Gottrons papules. Severe muscle wasting in both upper and lower limbs was seen. Gowers sign was positive. Extensive calcifications were seen in bilateral axillary region, around knee, hip and occipital region. ANA (Antinuclear Antibody) test by indirect immunofluorescence on Hep 2 cells showed speckled 2+ positivity (1:80). EMG (Electromyogram) and MRI (Magnetic Resonance Imaging) findings were consistent with dermatomyositis. Child was started on high dose corticosteroids along with oral methotrexate and hydroxychloroquin. Child improved and muscle strength improved. Subsequently child got readmitted in the next 6 months multiple times with cellulitis. Each time Staph aureus was obtained on aspiration and child responded to intravenous vancomycin. Child was started on Pamidronate infused intravenously at 1 mg/kg/day for 3 consecutive days every 3 months. Inaddition child received intravenous immunoglobulin monthly for 6 months. On follow-up after one year, there was complete clearance of calcinosis at all sites and good disease control without signs of myositis. Thus it appears that multiple drugs can resolve extensive calcinosis when used in combination.