Endocrine Abstracts

Introduction: Juvenile dermatomyositis may be associated with advanced calcinosis but the mechanism that leads to their development remains unclear.

Case report: A female patient admitted to the hospital at the age of 17 for skin rash, malaise and pain in peripheral joints and muscles. Congenital bilateral hip dysplasia. After evaluation a syndrome overlap with dominant atypical dermatomyositis and SLE was suspected and treatment with prednisolone and choroquine initiated. Six months after, subcutaneous calcium deposits appeared, first in gluteal region, than polytopically. No metabolic disorders were observed; levels of PTH, phosphorus and calcium have always been in reference range, ANA and anti-dsDNA occasionally elevated.

During the last 12 years, different immunosuppressive treatments: methotrexate, cyclophosphamide, azathioprin, mycophenolat, colchicine, intravenous bisfosfonates, imunoglobulins and calcium antagonists were given in attempt to stop spreading and reduce a calcinosis but without any improvement.

Presently, patient has a Gottrons papules on MCP and PIP joints of both hands. A very prominent extensive subcutaneous calcinosis with a few skin ulcers (from which calcium is leaking) is over thorax, abdomen, proximal parts of both hands, legs and pelvis, emphasized in lateral part of a right hip. The hands, distal parts of both legs and feet are spared. The muscles are atrophic and thin. Third muscle biopsy showed no calcifications but an increased number of macrophages in the perimysium, a few CD3 T-cells in the endomysial areas.

According to the early onset and a widespread calcinosis, a diagnosis of juvenile dermatomyositis is established. Last year, Hashimoto thyreoiditis and interstitial lung disease are diagnosed.

Sedimentation rate remains elevated which, with a current health condition, despite the prolonged treatment, indicates that disease is in active phase. Lastly, a treatment with abatacept (alternatively rituximab) was recommended.

Conclusion: Revealing a pathogenesis of calcium deposits would certainly bring a new insight to this case.