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Vol. 52. Núm. 5.
Páginas 424-429 (Mayo 2000)
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Vol. 52. Núm. 5.
Páginas 424-429 (Mayo 2000)
Acceso a texto completo
Dermatomiositis en la infancia
Dermatomyositis in childhood
Visitas
14950
M.E. Mateos Gonzáleza,*, E. López-Lasoa, J. Gómez Reinob, R. Simón de las Herasa, F. Mateos Beatoa
a Unidad de Neurología Pediátrica.
b Unidad de Reumatología Pediátrica. Departamento de Pediatría. Hospital 12 de Octubre. Madrid.
Este artículo ha recibido
Información del artículo
Objetivo

Análisis descriptivo de los datos clínicos, exploraciones complementarias y régimen terapéutico de los pacientes diagnosticados de dermatomiositis juvenil en nuestro centro, un hospital terciario.

Métodos

Estudio retrospectivo de las historias clínicas de los niños con diagnóstico definitivo de dermatomiositis juvenil seguidos en la consulta de reumatología pediátrica de nuestro centro, desde 1986 hasta julio de 1999.

Resultados

Del total de 9 casos recogidos, 3 han sido varones y 6 mujeres. La edad media al diagnóstico fue de 7 años. Los síntomas y signos que demandaron atención hospitalaria fueron: debilidad y dolor muscular asociados a síndrome constitucional en 4 casos (44%); debilidad muscular aislada en 2 casos; dolor muscular y síndrome constitucional en un caso, y monoartritis en otro. Un paciente presentó clínica exclusivamente cutánea al inicio. Presentaron calcinosis 3 pacientes. En 8 casos se produjo una elevación de la creatinfosfocinasa sérica y la lactatodeshidrogenasa en 8 pacientes; en 7 casos, una elevación de la aldolasa, y en 6 casos, en 8 pacientes un aumento de las aminotransferasas. Los autoanticuerpos fueron negativos en todos los pacientes. El electromiograma objetivó un patrón miopático o mixto en los 5 casos en que se realizó. La biopsia muscular fue diagnóstica en todos los casos. Siete pacientes recibieron tratamiento con corticoides y 2 pacientes corticoides, metotrexato y gammaglobulina. En cuanto a la evolución, 6 pacientes se encuentran asintomáticos, en 2 persiste debilidad muscular leve aislada y un paciente ha fallecido.

Conclusiones

Los resultados en nuestra serie concuerdan con la bibliografía. La dermatomiositis juvenil debe ser sospechada en niños con debilidad muscular y afectación del estado general. Se recomienda comenzar precozmente el tratamiento con corticoides.

Palabras clave:
Dermatomiositis juvenil
Niños
Dermatomiositis-tratamiento
Miopatías inflamatorias
Objective

The aim of this study is to review the presenting signs and symptoms, laboratory findings and therapeutic regimens of juvenile dermatomyositis in a tertiary hospital.

Methods

We reviewed retrospectively the available medical records of patients who met the clinicopathologic criteria of Bohan and Peter for definite juvenile dermatomyositis. They were followed between 1986 and july 1999 at the pediatric rheumatology section at our institution.

Results

The patient population included 3 male and 6 female patients. The mean age at diagnosis was 7 years. Clinical features demanding medical attention at the hospital were: muscle weakness and pain, with associated general symptoms in 4 cases; isolated muscle weakness in 2 cases; muscle weakness associated to general symptoms in 1 case; and monoarthritis in another case. One patient presented initially only with cutaneous rash. Three patients developed calcinosis. Serum CPK and LDH levels were elevated in 8 patients, aldolase in 7 and aminotransferases in 6. Autoantibodies were undetectable in all the patients. Electomyography demonstrated myopatic or mixed pattern in the 5 patients it was practiced. Muscle biopsy showed features of inflammatory myopathy in all the cases. Seven patients were treated exclusively with steroids and 2 patients with steroids, methotrexate and intravenous gammaglobuline. Actually, 6 patients are asymptomatic, 2 have mild muscle weakness, and 1 has died.

Conclusions

The results of our review agree with other series reported. Juvenile dermatomyositis suspicious should be made upon muscle weakness and general symptoms.

Treatment with steroids should be started promptly.

Key words:
Juvenile Dermatomyositis
Children
Dermatomyositisdrug therapy
Inflammatory myopathies
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