Clinical Images: Extensive calcinosis in lupus panniculitis



Illustration 1.

The patient, a 29-year-old woman with a 12-year history of systemic lupus erythematosus, was referred to the rheumatology department for treatment of lupus skin manifestations that had been resistant to combination treatment with multiple immunosuppressive agents and low-dose corticosteroids but responsive to increasing doses of corticosteroids. Her major skin manifestations were alopecia, discoid lupus lesions on the head, lupus panniculitis involving the upper arms, the shoulders, the breasts (also known as lupus mastitis), the buttocks, and the thighs, and chilblain lupus. Physical examination revealed multiple well-circumscribed, mobile, and mildly tender subcutaneous nodules with atrophy and inflammatory signs, on the skin of body areas affected by lupus panniculitis. The patient's current daily immunosuppressive treatment was mycophenolate mofetil 2 gm, hydroxychloroquine 400 mg, and prednisolone 10 mg. Mammography showed multiple coarse, dystrophic calcifications on both breasts, suggesting fat necrosis, a mammographic finding that has been previously reported in women with lupus mastitis. Three-dimensional computed tomography revealed extensive calcifications on the subcutaneous tissue of both breasts (A) and buttocks (B), with less extensive involvement at the shoulders and thighs, as a result of late-stage lupus panniculitis. Due to adverse effects the patient had experienced from prolonged use of corticosteroids, including an earlier history of osteonecrosis of both hips with total arthroplasty and recent osteonecrosis of the left talus, 2 doses of rituximab (1 gm) were administered 2 weeks apart. Over the next 2 months the alopecia, chilblain lupus, and discoid lupus lesions improved significantly and the tenderness was substantially reduced, but there was no change in the size of the subcutaneous nodules of lupus panniculitis. Her skin manifestations remained under control with hydroxychloroquine 400 mg and prednisolone 5 mg daily. After 1 year, a new flare of the skin disease was treated successfully with a second course of rituximab.