Calcinosis universalis in juvenile dermatomyositis

¹Pediatrics Department Charles Nicolle Hospital of Tunis, Tunis, Tunisia, ²University of Tunis El Manar, Tunis, Tunisie, ³Internal Medicine Department “A”, Charles Nicolle Hospital, Tunis, Tunisia, ⁴Research Laboratory of Kidney Diseases, Charles Nicolle Hospital of Tunis, Tunis, Tunisia

^&Corresponding author
Mounira El Euch, Internal Medicine Department “A”, Charles Nicolle Hospital, Tunis, Tunisia

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A 5 years-old female Tunisian child was admitted in our department for inflammatory polyarthralgia, progressive limb weakness, severe muscle fatigability and fever. On physical examination she had heliotrope eyelid edema, extensive calcalreous depositis in her four extremities, trunk, abdomen and on the back with Gottron' papules. Muscle enzymes were high particularly Creatinine Phosphokinase (CPK). Electromyography showed a myogenic process and muscle biopsy revealed characteristic signs of inflammatory muscle disease. Radiographic studies revealed extensive deep muscular calcareous deposits regarding joints. Juvenile Dermatomyositis (DM) complicated with calcinosis universalis was retained. Although intensive glucocorticoids combined with methotrexate was given, the calcific nodules gradually increased in size, ulcerated frequently and restricted her joint mobility. This case illustrates an extreme rare form of calcinosis which occurs more commonly in juvenile DM than in adult DM. There is until now no successful treatment of calcinosis universalis identified to date. Few reports described successful treatment with immunoglobulins, probenecid, aluminum hydroxide, warfarin, and diltiazem. Further investigation of disease mechanisms is needed so more effective therapeutic strategies may be developed.