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Cutaneous Calcinosis in Juvenile Dermatomyositis
Cutaneous Calcinosis in Juvenile Dermatomyositis
A
22-year-old man presented with multiple cutaneous calcinosis and ulceration on his buttocks. He had been diagnosed with juvenile dermatomyositis (JDM) 10 years earlier, for which he had been treated with high-dose prednisolone, high-dose intravenous immunoglobulin, and immunosuppressive drugs including cyclosporine, azathioprine, mycophenolate mofetil, and tacrolimus. However, symptoms relapsed with prednisolone weaning. Rituximab was effective for skin manifestations and muscle weakness, but the calcinosis remained unchanged. Physical examination revealed a tender, firm nodule with central ulceration surrounded by erythematous skin, measuring up to 50 mm in diameter at his buttocks. Radiologic examination with 3-dimensional computed tomography (CT) clearly demonstrated numerous superficial and deep calcium deposits. Large tumorous deposits of calcium, which appear as popcorn-like areas, were observed on the patient’s buttocks (Figure). These large deposits were surgically removed. Calcinosis occurs in 30%-70% of children with JDM.1 It usually occurs late in the course of the disease. The known
risk factors for calcinosis include delayed treatment and severe disease.2 Although little is known about the pathophysiology of the calcinosis of JDM, it is supposed that calcinosis may be mediated by activated macrophages and chronic local inflammation might be closely associated with the development of calcinosis.3 In our patient, calcinosis was observed over the entire body; however, large tumorous deposits of calcium were observed on the buttocks, a predilection site of trauma. This indicates that calcinosis can occur at any site, but the enlargement of deposits might be associated with chronic inflammation on recurrent traumatic stress. Imaging of soft-tissue calcifications typically begins with conventional radiography. CT has been reported to be more specific and sensitive than conventional radiography in the detection of deeper soft-tissue calcifications.4 Threedimensional CT has the added advantage of depicting ossifying lesions and the exact plane of calcification. n Masaki Shimizu, MD, PhD Kazuyuki Ueno, MD Sayaka Ishikawa, MD Tadafumi Yokoyama, MD, PhD Yoshihito Kasahara, MD, PhD Akihiro Yachie, MD, PhD Department of Pediatrics School of Medicine Institute of Medical, Pharmaceutical, and Health Sciences Kanazawa University Kanazawa, Japan
References
Figure. Three-dimensional CT scan of the patient.
1. Packman LM. Juvenile dermosynovitis, pathophysiology and disease expression. Pediatr Clin North Am 1995;42:1071-98. 2. Ansell BM. Juvenile dermatomyositis. Rheum Dis Clin North Am 1991; 17:931-42. 3. Mukamel M, Horev G, Mimouni M. New insight into calcinosis of juvenile dermatomyositis: a study of composition and treatment. J Pediatr 2001;138:736-66. 4. Fishel B, Diamant S, Papo I, Yaron M. CT assessment of calcinosis in a patient with dermatomyositis. Clin Rheumatol 1986;5:242-4.
J Pediatr 2013;163:921. 0022-3476/$ - see front matter. Copyright ª 2013 Mosby Inc. All rights reserved. http://dx.doi.org/10.1016/j.jpeds.2013.03.048
921
A
22-year-old man presented with multiple cutaneous calcinosis and ulceration on his buttocks. He had been diagnosed with juvenile dermatomyositis (JDM) 10 years earlier, for which he had been treated with high-dose prednisolone, high-dose intravenous immunoglobulin, and immunosuppressive drugs including cyclosporine, azathioprine, mycophenolate mofetil, and tacrolimus. However, symptoms relapsed with prednisolone weaning. Rituximab was effective for skin manifestations and muscle weakness, but the calcinosis remained unchanged. Physical examination revealed a tender, firm nodule with central ulceration surrounded by erythematous skin, measuring up to 50 mm in diameter at his buttocks. Radiologic examination with 3-dimensional computed tomography (CT) clearly demonstrated numerous superficial and deep calcium deposits. Large tumorous deposits of calcium, which appear as popcorn-like areas, were observed on the patient’s buttocks (Figure). These large deposits were surgically removed. Calcinosis occurs in 30%-70% of children with JDM.1 It usually occurs late in the course of the disease. The known
risk factors for calcinosis include delayed treatment and severe disease.2 Although little is known about the pathophysiology of the calcinosis of JDM, it is supposed that calcinosis may be mediated by activated macrophages and chronic local inflammation might be closely associated with the development of calcinosis.3 In our patient, calcinosis was observed over the entire body; however, large tumorous deposits of calcium were observed on the buttocks, a predilection site of trauma. This indicates that calcinosis can occur at any site, but the enlargement of deposits might be associated with chronic inflammation on recurrent traumatic stress. Imaging of soft-tissue calcifications typically begins with conventional radiography. CT has been reported to be more specific and sensitive than conventional radiography in the detection of deeper soft-tissue calcifications.4 Threedimensional CT has the added advantage of depicting ossifying lesions and the exact plane of calcification. n Masaki Shimizu, MD, PhD Kazuyuki Ueno, MD Sayaka Ishikawa, MD Tadafumi Yokoyama, MD, PhD Yoshihito Kasahara, MD, PhD Akihiro Yachie, MD, PhD Department of Pediatrics School of Medicine Institute of Medical, Pharmaceutical, and Health Sciences Kanazawa University Kanazawa, Japan
References
Figure. Three-dimensional CT scan of the patient.
1. Packman LM. Juvenile dermosynovitis, pathophysiology and disease expression. Pediatr Clin North Am 1995;42:1071-98. 2. Ansell BM. Juvenile dermatomyositis. Rheum Dis Clin North Am 1991; 17:931-42. 3. Mukamel M, Horev G, Mimouni M. New insight into calcinosis of juvenile dermatomyositis: a study of composition and treatment. J Pediatr 2001;138:736-66. 4. Fishel B, Diamant S, Papo I, Yaron M. CT assessment of calcinosis in a patient with dermatomyositis. Clin Rheumatol 1986;5:242-4.
J Pediatr 2013;163:921. 0022-3476/$ - see front matter. Copyright ª 2013 Mosby Inc. All rights reserved. http://dx.doi.org/10.1016/j.jpeds.2013.03.048
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