Clinical Image: Dystrophic Calcinosis in Systemic Sclerosis–Systemic Lupus Erythematosus Overlap Syndrome

The patient, a 40-year-old woman, presented with 3-month progressive swelling in the cubital fossa without recall of a causative event. She had a 17-year history of systemic sclerosis–systemic lupus erythematosus overlap syndrome treated with iloprost. Years before, after a pregnancy, she underwent amputation of the distal phalanges as a result of painful Raynaud phenomenon complicated with digital necrosis. On physical examination, the swelling in the cubital fossa was painless, elastic, mildly mobile, and not adhered to the overlying skin. Radiographs (lateral radiograph shown on the left) revealed large, mixed nodular, and flock-like calcium deposits within the soft tissues of the anterior aspect of the arm, bulging on the skin contour, in keeping with dystrophic calcifications. Further characterization of the extension of soft tissue involvement and surrounding inflammatory change was performed with magnetic resonance imaging. The sagittal proton density weighted spectral attenuated inversion recovery image (PDW-SPAIR, on the right) demonstrates the calcification as grossly hypointense, involving the superficial plane (hypodermis – subcutaneous fatty tissue) and extending in depth to involve the superficial aspect of the distal biceps brachialis, and brachialis muscle bellies and tendons, to their insertions (Note inhomogeneous fat saturation in the olecranon as a result of positioning limitation because of the inability to fully extend the elbow). The main differential is myositis ossificans, which is seen in the context of previous trauma and tends to have an "eggshell-like" appearance with a lucent center. There is normally a radiolucent cleft (string sign) separating the lesion from the cortex of the adjacent bone. Dystrophic calcinosis with overlap syndrome is uncommon, and its pathogenesis is still not fully understood. Hypothesis are chronic tissue inflammation, structural defects, and hypoxemia. The condition develops around major joints, such as elbows and knees. No drug therapy has been proven to be effective for calcinosis, although some novel therapies have recently been suggested. Options for management include treatment of the underlying cause, in the cases of secondary calcinosis, anti-inflammatory drugs to palliate symptoms, and antibiotic therapy in case of superimposed infection. Surgical debulking may be helpful in cases of restricted function. Recently, local and minimally invasive therapies, such as laser therapy, extracorporeal shockwave lithotripsy, topical sodium thiosulfate, and intralesional sodium thiosulfate have been suggested as potential lines of treatment, but evidence is insufficient to prove their efficacy. Disclosure Form: Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article.