A case of a generalized symptomatic calcinosis in systemic sclerosis

Abstract

Calcinosis consists of abnormal calcium deposition in soft tissues, which appears often in patients with limited systemic sclerosis, being one of the criteria of CREST (calcinosis cutis, Raynaud phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia) syndrome. With a long evolution, the aim of the treatment is to control the symptoms and prevent complications. In this article, we present the challenging management of a profuse lesion of calcinosis in a patient with systemic sclerosis. We describe the case of a 52-year-old woman with systemic sclerosis and CREST syndrome who was admitted in our Department with multiple painful and disabling tumoral masses, situated in nearly all joints. The interscapular vertebral tumoral mass was excised and the defect was closed. Histopathological examination revealed cutaneous calcinosis, probably associated with CREST syndrome, a type of scleroderma. Postoperative results were favorable and no local complications were encountered. Six months follow-up revealed no evidence of recurrence. Despite the size and the invasion of the tumor in the muscle, complete resection was possible with an adequate reconstruction; the postoperative result being acceptable. With a lower response to medication, surgical treatment is considered the only option for treating symptomatic lesions of calcinosis in order to improve quality of life.