Juvenile dermatomyositis in the Nice area:: a retrospective study 1991-2001.

Introduction. juvenile dermatomyositis is a rare disease. We conducted a retrospective chart review on patients with juvenile dermatomyositis diagnosed in the Nice area from 1991 to 2001. Our purpose was to review diagnosis criteria and treatment strategies. Patients and methods. The cases of juvenile dermatomyositis were identified by phone investigation of physicians of the departments of paediatrics, dermatology, rheumatology and internal medicine. Results. Seven cases of juvenile dermatomyositis (sex ratio M/W: 0.75, medium age: 7.7 years) were identified. Myalgia and/or weakness were the main reasons for initial consultation. Cutaneous lesions were present in all patients on initial presentation. Muscular enzymes were abnormal in 4 cases out of 7. Muscular biopsy was conducted in 6 patients. In one case, MRI revealed an inflammatory involvement although no clinical or biological sign existed. Systemic corticosteroids was the initial treatment, associated with monthly intravenous. immunoglobulins in 5 cases, allowing initial control of the disease in all cases. The other therapies were: methotrexate (3 cases), ciclosporin A (1 case), and chloroquine (2 cases). The evolution was monocyclic in three cases, polycyclic in four cases. With a median follow-up of two years, all the patients are alive: five under treatment, three still in first flare. Comment. The diagnosis strategy seems relevant because at least 3 criteria of Peter and Bohan were found in 6/7 patients. Muscular biopsy appears fundamental in the diagnosis strategy. MRI was useful when it was conducted but its use has to be assessed. Therapies were those of previous published studies, except for the use of intravenous immunoglobulins as first-line treatment, associated with corticosteroids. This strategy may be justified for corticosteroid-sparing purposes. Conclusion. Working on a real, well-codified strategy of diagnosis and treatment would enhance the uniform management of these patients.