Extensive calcinosis cutis universalis in a patient with systemic lupus erythematosus: 10‐year treatment experience

We report the case of a 37-year-old woman with systemic lupus erythematosus (SLE), who spontaneously developed several isolated, hard, erythematous, and painful plaques with ulceration in her right lower leg 4 years after the diagnosis. The plaques then gradually developed into nodules with a hard base. She suffered from the progression of purulent and chalky, milk-like discharge from the ulcerative wounds, even with oral prednisolone treatment (the dose varied from 10 mg to 30 mg per day). The laboratory data showed normal serum calcium, phosphorus, parathyroid hormone, and muscle enzyme levels. Several episodes of cellulites were also noted over the lesion sites with various pathogens isolated. A plain film X-ray showed numerous small soft tissue calcifications extensively scattered over her pelvic region (Fig. 1A), thigh, knee, and lower leg (Fig. 1B). However, computed tomography revealed that the calcified lesions were only present in the skin and subcutaneous tissues, with no muscle, vessel, or visceral organ invasion (Fig. 1C). Pathology of the skin and subcutaneous tissue showed patches of black-colored staining, indicating tissue calcification under microscopy (Fig. 1D). The diagnosis was extensive calcinosis cutis universalis. During this period, she underwent surgery several times for the removal of the newly isolated calcified masses over her buttocks and four extremities. She also received bisphosphate 10 mg/kg/day for 6 months, ceftriaxone 2 g/day for 30 days, diltiazem 30 mg/ day for more than 1 year, and intralesional corticosteroids. However, the general condition still progressed. Calcinosis cutis is a rare disorder characterized by calcium deposition in the skin and subcutaneous tissues.