[Juvenile dermatomyositis: clinical manifestations and laboratory tests]. / Dermatomiositis juvenil: manifestaciones clínicas y de laboratorio.

ABSTRACT

Juvenile dermatomyositis (JDM) is a chronic, inflammatory degenerative entity with multisystemic involvement, particularly of skin and striated muscle. This is a description of the clinical features, laboratory findings and treatment results in 10 children with JDM which were under control along a 14 years period (1974-1988) in a general hospital at metropolitan Santiago, Chile. Onset was insidious in 8 cases and acute in the remaining patients. All children had muscle and skin involvement, 90% of them showed heliotrops erythema and 6 cases (among the total 10) had one or more additional skin signs. Ninety percent of patients had risen serum activity of muscle associated enzymes, lactic dehydrogenase (LDH) being the most constantly elevated 7/7 studied cases. All patients showed abnormal electromyographic and histologic findings in muscle biopsy. Treatment was based upon rehabilitation, prednisone (average daily dose 1.5 mg.kg of body weight) associated or not with cytotoxic drugs, accordingly to each individual case. Clinical course was considered to be favourable in 9 of the 10 cases because all of them showed no evidence of disease activity on last follow up control 26 to 93 months after initial consultation, six had only slight restriction to wider range movements of affected joints and three showed no sequelae. Only one death occurred in this series, to a patient who rejected treatment.