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Massive Gluteal Calcinosis in a 10-Year-Old Girl with Juvenile Dermatomyositis: Successful Surgical Management

Vitale, Agata, M.D., Ph.D.; Delia, Gabriele, M.D., Ph.D.; Torre, Francesco La, M.D.; Calcagno, Giuseppina; d'Alcontres, Francesco Stagno

Plastic and Reconstructive Surgery: December 2009 - Volume 124 - Issue 6 - p 456e-458e
doi: 10.1097/PRS.0b013e3181bcf703
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Department of Medical and Surgical Pediatric Sciences, Pediatric Rheumatology Unit (Vitale)

Department of Plastic and Reconstructive Surgery (Delia)

Department of Medical and Surgical Pediatric Sciences, Pediatric Rheumatology Unit (La Torre, Calcagno)

Department of Plastic and Reconstructive Surgery, Policlinico G. Martino, University of Messina, Messina, Italy (d'Alcontres)

Correspondence to Dr. Vitale, Department of Paediatrics, Rheumatology Unit, “G. Martino” Hospital, Pad. NI, Via Consolare Valeria, 98100 Messina, Italy, vitale@interfree.it

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Sir:

Calcinosis is a devastating complication of juvenile dermatomyositis and a challenging therapeutic problem. Juvenile dermatomyositis is a multisystemic autoimmune disease characterized by skin and muscle vasculitis. Its incidence is two to three cases per 1 million children per year.1 Calcinosis in juvenile dermatomyositis is dystrophic and occurs in one-third of patients. The gluteal region is a rare and dreadful location, causing severe functional and aesthetic problems in a young girl with juvenile dermatomyositis. Complete surgical excision was performed with a good outcome.

A 10-year-old Caucasian girl was referred to the department of pediatric rheumatology for evaluation of diffuse calcific nodules. She had been diagnosed as having juvenile dermatomyositis at the age of 7 years. Three years after diagnosis, the patient relapsed, with the appearance of multiple, firm, whitish nodules, some of which were located in both gluteal regions (Fig. 1). Routine blood tests showed normal serum calcium, inorganic phosphate, alkaline phosphatase, and parathyroid hormone. Combinations of prednisone and hydroxychloroquine were administered without improvement. The calcific painful nodules gradually increased in size and restricted her mobility. They were complicated by repeated bacterial infections resistant to medical therapy. On surgical evaluation, two subcutaneous ovular gluteal masses were detected with one fistula in the left region. The computed tomographic findings revealed no relationship with the surrounding muscles and bone. Under general anesthesia, en bloc wide excision of the masses with a functional reconstruction and gluteal remodeling was carried out. Histologic examination confirmed a dystrophic calcinosis. Antibiotic prophylaxis was administered and the patient had an uneventful postoperative evolution. Treatment with bisphosphonates and an immunosuppressant (methotrexate) was started 12 days after surgery. At 1-year-follow-up, the nodules had not recurred and the patient had not had infectious or healing complications resulting from vasculitis to which he was predisposed. An aesthetic gluteal contour was obtained (Fig. 2).

Fig. 1.

Fig. 1.

Fig. 2.

Fig. 2.

Four different patterns of dystrophic calcinosis have been described in patients with juvenile dermatomyositis: superficial plaques or nodules, deep nodular deposits that extend to the muscles, deposits along fascial planes of the muscles and tendons, and an extensive hard calcium deposit that covers the entire surface of the body. The sites frequently affected are the elbows, knees, digits, and extremities. It is believed that calcium salt deposits occur with severe juvenile dermatomyositis cases with persistent inflammation. Macrophages and proinflammatory cytokines have been observed in calcium fluids.2 Treatment is based on anecdotal reports.3 Bisphosphonates are a potentially promising approach through inhibition of calcium hydroxyapatite formation, macrophage function, and bone calcium resorption.4 Surgical removal is indicated for patients experiencing chronic pain, loss of function, infections, or nonhealing ulcers. Potential complications of surgery include problems with wound healing and recurrence.5 This tends to be uncommon with good control of the dermatomyositis before undertaking surgery; use of low doses of corticosteroids to minimize disruption to wound healing; and minimization of surgical trauma, avoiding healing by secondary intention. In conclusion, surgical resection with attention to detail with concomitant specific medical therapy is recommended for treatment of gluteal calcinosis in juvenile dermatomyositis.

Agata Vitale, M.D., Ph.D.

Department of Medical and Surgical Pediatric Sciences

Pediatric Rheumatology Unit

Gabriele Delia, M.D., Ph.D.

Department of Plastic and Reconstructive Surgery

Francesco La Torre, M.D.

Giuseppina Calcagno

Department of Medical and Surgical Pediatric Sciences

Pediatric Rheumatology Unit

Francesco Stagno d'Alcontres

Department of Plastic and Reconstructive Surgery

Policlinico G. Martino

University of Messina

Messina, Italy

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DISCLOSURE

The authors received no support from any company or community for preparation of this article.

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REFERENCES

1. Feldman BM, Rider LG, Reed AM, Pachman LM. Juvenile dermatomyositis and other idiopathic inflammatory myopathies of childhood. Lancet 2008;371:2201–2212.
2. Marhaug G, Shah V, Shroff R, et al. Age-dependent inhibition of ectopic calcification: A possible role for fetuin-A and osteopontin in patients with juvenile dermatomyositis with calcinosis. Rheumatology (Oxford) 2008;47:1031–1037.
3. Riley P, McCann LJ, Maillard SM, Woo P, Murray KJ, Pilkington CA. Effectiveness of infliximab in the treatment of refractory juvenile dermatomyositis with calcinosis. Rheumatology (Oxford) 2008;47:877–880.
4. Ambler GR, Chaitow J, Rogers M, McDonald DW, Ouvrier RA. Rapid improvement of calcinosis in juvenile dermatomyositis with alendronate therapy. J Rheumatol. 2005;32:1837–1839.
5. Kerstein MD. The non-healing leg ulcer: Peripheral vascular disease, chronic venous insufficiency, and ischemic vasculitis. Ostomy Wound Manage. 1995;42(10A Suppl):19S–35S.

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