Systemic sclerosis sine scleroderma and calcinosis cutis: report of a rare case

Systemic sclerosis sine scleroderma is a rare form of limited cutaneous scleroderma. These patients manifest without cutaneous involvement, but do not differ in its clinical or laboratory features and prognosis from classical systemic sclerosis. In the absence of cutaneous signs/symptoms, its diagnosis is delayed leading to morbidity. The exact prevalence of dystrophic calcification in systemic sclerosis sine scleroderma, though a feature of systemic sclerosis, is not known. Development of dystrophic calcification further aggravates patient's woes. This paper describes systemic sclerosis sine scleroderma in a 17-year-old girl diagnosed initially as seronegative juvenile rheumatoid arthritis. She developed progressively increasing disk-like masses of calcinosis over the gluteal regions, knee, elbow, and ankle joints fixed to the overlying skin associated with malaise, anorexia, and weight loss. There was no Raynaud's phenomenon, dysphagia, dyspnoea, sclerodermatous skin, sclerodactyly, telangectasias, or muscle tenderness/weakness. Digital pitted scars, elevated anticentromere antibody values, esophageal hypomotility, and fluffy calcification in subdermal soft tissues in gluteal regions and around wrist, hip, knees, heels, and ankle joints (without affecting the underlying structures) were detected. Therapy with diltiazem and magnesium/aluminum antacids was useful in resolving calcinosis.