Mustafa ÇAKAN, Şerife Gül KARADAĞ, Nuray Aktay AYAZ

Complete and sustained resolution of calcinosis universalis in a juvenile dermatomyositis case with mycophenolate mofetil

Juvenile dermatomyositis (JDM) is a rare, multisystemic, idiopathicvasculopathy mainly affecting the muscles and the skin. Gastrointestinalsystem, lungs, joints and heart may also be involved. Characteristic skin findingsare heliotrope rash and Gottron papules but extensive skin involvement aslarge necrotic lesions are rarely reported. Calcinosis is one of the major issuesin the long term. Delay in diagnosis, inadequate therapy at the initial phase,prolonged persistent disease activity are considered as major risk factors forthe development of calcinosis. Treatment of calcinosis is also a major issuebecause no single treatment modality has been found to reproducibly stop orreverse calcification.A 5-year-old girl was admitted to our clinic with typical signs and symptomsof JDM. She was initially treated with high-dose corticosteroids, methotrexateand intravenous immunoglobulin (IVIG). Soon after, she developed necroticulcerative skin lesions and cyclosporine was added to her treatment regimen.By this treatment all muscle and skin manifestations were controlled but onthe first year of follow-up she developed superficial calcification plaques onthe upper extremities and calcinosis universalis like calcifications on the lowerextremities. Calcifications did not respond to bisphosphonate (pamidronate)and IVIG treatment but mycophenolate mofetil resulted in rapid and sustainedresolution of all calcification plaques.

PDF

___

1. Rider LG, Lindsey CB, Miller FW. Juvenile Dermatomyositis. In: Petty RE, Laxer RM, Lindsey CB, Wedderburn LR (eds). Textbook of Pediatric Rheumatology (7th ed). Philadelphia: Elsevier, 2016: 351-383.

2. Huber AM. Idiopathic inflammatory myopathies in childhood: Current concepts. Pediatr Clin North Am 2012; 59: 365-380.

3. Enders FB, Bader-Meunier B, Baildam E, et al. Consensus-based recommendations for the management of juvenile dermatomyositis. Ann Rheum Dis 2017; 76: 329-340.

4. Huber AM, Robinson AB, Reed AM, et al; Members of the Juvenile Dermatomyositis Sub-Committee of the Children's Arthritis and Rheumatology Research Alliance Consensus treatments for moderate juvenile dermatomyositis: beyond the first two months. Results of the second Childhood Arthritis and Rheumatology Research Alliance Consensus Conference. Arthritis Care Res (Hoboken) 2012; 64: 546-553.

5. Bohan A, Peter JB. Polymyositis and dermatomyositis. N Eng J Med 1975; 292: 344-347.

6. Papadopoulou C, McCann LJ. The vasculopathy of juvenile dermatomyositis. Front Pediatr 2018; 6: 284.

7. Schmeling H, Stephens S, Goia C, et al. Nailfold capillary density is importantly associated over time with muscle and skin disease activity in juvenile dermatomyositis. Rheumatology (Oxford) 2011; 50: 885-893.

8. Kim S, El-Hallak M, Dedeoglu F, Zurakowski D, Fuhlbrigge RC, Sundel RP. Complete and sustained remission of juvenile dermatomyositis resulting from aggressive treatment. Arthritis Rheum 2009; 60: 1825-1830.

9. Shimojima Y, Ishii W, Kato T, et al. Intractable skin necrosis and interstitial pneumonia in amyopathic dermatomyositis successfully treated with cyclosporin A. Intern Med 2003; 42: 1253-1258.

10. Hoetzel MF, Oberle EJ, Robinson AB, Agarwal A, Rider LG. The presentation, assessment, pathogenesis, and treatment of calcinosis in juvenile dermatomyositis. Curr Rheumatol Rep 2014; 16: 467.

11. Tansley SL, Betteridge ZE, Shaddick G, et al; Juvenile Dermatomyositis Research Group. Calcinosis in juvenile dermatomyositis is influenced by both antiNXP2 autoantibody status and age at disease onset. Rheumatology (Oxford) 2014; 53: 2204-2208.

12. Demirkaya E, Bilginer Y, Aktay-Ayaz N, et al. Neuropsychiatric involvement in juvenile systemic lupus erythematosus. Turk J Pediatr 2008; 50: 126- 131.

13. Dagher R, Desjonqueres M, Duquesne A, et al. Mycophenolate mofetil in juvenile dermatomyositis: a case series. Rheumatol Int 2012; 32: 711-716.

14. Rouster-Stevens KA, Morgan GA, Wang D, Pachman LM. Mycophenolate mofetil, a possible therapeutic agent for children with juvenile dermatomyositis. Arthritis Care Res (Hoboken) 2010; 62: 1446-1451.