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Ectopic Tumoral Calcinosis in Systemic Sclerosis
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Ectopic Tumoral Calcinosis in Systemic Sclerosis Thierry Zenone, MD* and Marie Couret, MD *Department of Medicine, Unit of Internal Medicine and Rheumatology, Centre Hospitalier General, Valence, France (E-mail: [email protected]) The authors have no financial or other conflicts of interest to disclose.
FIGURE 2 FIGURE 1
A
66-year-old woman received a diagnosis of limited cutaneous systemic sclerosis (formerly CREST syndrome: Calcinosis, Raynaud’s phenomenon, Esophageal dysmotility, Sclerodactyly, Telangiectasia) 10 years previously because of arthralgias, synovitis, finger stiffness, Raynaud’s phenomenon and anticentromere antibodies. Subcutaneous hard lumps developed over her fingers, elbows and buttocks and progressed clinically and radiographically. Radiography showed soft tissue calcifications of the fingers (Figure 1). The biggest lesions were subsequently excised, but they reappeared rapidly after surgery. She had low-back pain leading to discovery of a voluminous ectopic tumoral calcinosis of the spine seen on radiography (Figure 2). Results of blood and urinary phosphocalcium analyses were normal. Since diagnosis, the patient’s treatment has included glucocorticoids and methotrexate for systemic sclerosis, and colchicine for calcinosis. Despite treatment, the patient has persistent, extensive calcification over her fingers, elbows and buttocks, with recurrent ulcerations and infections at calcified sites. The patient had progressing diffuse disease with pulmonary fibrosis. She was treated with monthly intravenous
The American Journal of the Medical Sciences
cyclophosphamide and deceased 1 year later because of cardiac insufficiency. Calcinosis has been reported in cases of systemic sclerosis, mixed connective-tissue disorders, systemic lupus erythematosus, dermatomyositis and antisynthetase syndrome. The condition can cause substantial complications (skin ulceration, recurrent episodes of local inflammation and infection) and is difficult to treat.1 Although subcutaneous and periarticular calcifications limited in size are frequent in patients with systemic sclerosis (about 25% of patients, mainly CREST syndrome), there have been a few reports of tumoral calcifications involving the spine.1,2 Ectopic tumoral calcinosis might reflect an advanced stage of systemic sclerosis with circulatory disturbance and extensive fibrosis. REFERENCES 1. Boulman N, Slobodin G, Rozenbaum M, et al. Calcinosis in rheumatic diseases. Semin Arthritis Rheum 2005;34:805–12. 2. Nagai Y, Sogabe Y, Ishikawa O. Tumoral calcinosis of the ribs and lumbar spine in systemic sclerosis. Eur J Dermatol 2008;18:473–74.
Volume 349, Number 6, June 2015
e7
IN THE
MEDICAL SCIENCES
Ectopic Tumoral Calcinosis in Systemic Sclerosis Thierry Zenone, MD* and Marie Couret, MD *Department of Medicine, Unit of Internal Medicine and Rheumatology, Centre Hospitalier General, Valence, France (E-mail: [email protected]) The authors have no financial or other conflicts of interest to disclose.
FIGURE 2 FIGURE 1
A
66-year-old woman received a diagnosis of limited cutaneous systemic sclerosis (formerly CREST syndrome: Calcinosis, Raynaud’s phenomenon, Esophageal dysmotility, Sclerodactyly, Telangiectasia) 10 years previously because of arthralgias, synovitis, finger stiffness, Raynaud’s phenomenon and anticentromere antibodies. Subcutaneous hard lumps developed over her fingers, elbows and buttocks and progressed clinically and radiographically. Radiography showed soft tissue calcifications of the fingers (Figure 1). The biggest lesions were subsequently excised, but they reappeared rapidly after surgery. She had low-back pain leading to discovery of a voluminous ectopic tumoral calcinosis of the spine seen on radiography (Figure 2). Results of blood and urinary phosphocalcium analyses were normal. Since diagnosis, the patient’s treatment has included glucocorticoids and methotrexate for systemic sclerosis, and colchicine for calcinosis. Despite treatment, the patient has persistent, extensive calcification over her fingers, elbows and buttocks, with recurrent ulcerations and infections at calcified sites. The patient had progressing diffuse disease with pulmonary fibrosis. She was treated with monthly intravenous
The American Journal of the Medical Sciences
cyclophosphamide and deceased 1 year later because of cardiac insufficiency. Calcinosis has been reported in cases of systemic sclerosis, mixed connective-tissue disorders, systemic lupus erythematosus, dermatomyositis and antisynthetase syndrome. The condition can cause substantial complications (skin ulceration, recurrent episodes of local inflammation and infection) and is difficult to treat.1 Although subcutaneous and periarticular calcifications limited in size are frequent in patients with systemic sclerosis (about 25% of patients, mainly CREST syndrome), there have been a few reports of tumoral calcifications involving the spine.1,2 Ectopic tumoral calcinosis might reflect an advanced stage of systemic sclerosis with circulatory disturbance and extensive fibrosis. REFERENCES 1. Boulman N, Slobodin G, Rozenbaum M, et al. Calcinosis in rheumatic diseases. Semin Arthritis Rheum 2005;34:805–12. 2. Nagai Y, Sogabe Y, Ishikawa O. Tumoral calcinosis of the ribs and lumbar spine in systemic sclerosis. Eur J Dermatol 2008;18:473–74.
Volume 349, Number 6, June 2015
e7