Background: Calcinosis cutis is usually seen in long standing and untreated cases of juvenile dermatomyositis.
Case characteristics: 7-year-old girl with severe calcinosis cutis who developed cutaneous ulceration, rash and myopathy.
Observation: Myopathic changes in EMG, muscle edema in MRI, elevated muscle enzymes and Jo-1 positive antibodies.
Outcome: Treatment with prednisolone and methotrexate resulted in improvement of the lesion.
Message: Calcinosis cutis may be a presenting feature of juvenile dermatomyositis even in the absence of characteristic findings of rash and weakness.