Abstract

PANDOLFO, SOLEDAD; CAGGIANI, MARINA  and  RUBIO, IVONNE. Dermatomiositis juvenil.: A propósito de un caso con grave compromiso multisistémico. Arch. Pediatr. Urug. [online]. 2005, vol.76, n.4, pp.305-311. ISSN 1688-1249.

A fourteen year old boy with an unusual presentation of juvenile dermatomyositis (JDM) is described. The illness was characterized by cutaneous manifestations like heliotrope eyelids, Gottron papules and proximal muscle weakness. Elevated serum levels of muscle-derived enzymes and the characteristic electromyogram completed the diagnosis of JDM. Respiratory assistance was needed due to the severe respiratory failure. Multisystemic failure involving the central nervous system, gastrointestinal tract and hematopoyetic system occurred. Aggressive immunosupression with corticosteroids, ciclophosphamide and intravenous immunoglobulin leaded to a favorable outcome. The disease has been in remission for 18 months with low doses of corticosteroids and methotrexate.

Keywords : DERMATOMYOSITIS-diagnosis; DERMATOMYOSITIS-therapy; DERMATOMYOSITIS-complications.

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