Información de la revista
Vol. 52. Núm. 5.
Páginas 424-429 (Mayo 2000)
Compartir
Compartir
Descargar PDF
Más opciones de artículo
Vol. 52. Núm. 5.
Páginas 424-429 (Mayo 2000)
DOI: 10.1016/S1695-4033(00)77374-7
Acceso a texto completo
Dermatomiositis en la infancia
Dermatomyositis in childhood
Visitas
...
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.
Información del artículo
Resumen
Bibliografía
Descargar PDF
Estadísticas
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
El Texto completo está disponible en PDF
Biblografía
[1.]
B.A. DeNardo, L.B. Tucker, L.C. Miller, I.S. Szer, J.G. Schaller.
Demography of a regional pediatric rheumatology patient population.
J Rheumatol, 21 (1994), pp. 1553-1561
[2.]
L.G. Rider, F.W. Miller.
Classification and treatment of the juvenile idiopathic inflammatory miopathies.
Rheum Dis ClinNorth Am, 23 (1997), pp. 619-646
[3.]
A Bohan, JB Peter.
Polymyositis and dermatomyositis: I y II.
N Engl J Med, 292 (1975), pp. 344-347
[4.]
L.M. Pachman, M.L. Maryjowski.
Juvenile dermatomyositis and polymyositis.
Clin Rheum Dis, 10 (1984), pp. 95-115
[5.]
L.M. Pachman.
Juvenile dermatomyositis. Pathophysiology and disease expression.
Pediatr Clin North Am, 42 (1995), pp. 1071-1096
[6.]
V. Dubowitz.
Inflammatory myopathies: juvenile dermatomyositis.
Muscle disorders in childhood, 2.a, pp. 427-447
[7.]
T. Hiketa, Y. Matsumoto, M. Ohashi, R. Sasaki.
Juvenile dermatomyositis: a statistical study of 114 patients with dermatomyositis.
J Dermatol, 19 (1992), pp. 470-476
[8.]
M.A.J. Van Rossum, I. Hiemstra, A.M. Prieur, G.T. Rijkers, Kuis.
Juvenile dermato/polymyositis: a retrospective analysis of 33 cases with special focus on initial CPK levels.
Clin Exp Rheumatol, 12 (1994), pp. 339-342
[9.]
L.M. Pachman, J.R. Hayford, M.C. Hochberg, M.A. Pallansch, A. Chung, C.D. Daugherty.
New-onset juvenile dermatomyositis.
[10.]
D.M. Eisenstein, A.S. Paller, L.M. Pachman.
Juvenile dermatomyositis presenting with rash alone.
Pediatrics, 100 (1997), pp. 391-392
[11.]
L.M. Pachman, J.M. Friedman, M.L. Maryjowski-Sweeney, O. Jonnasson, R.M. Radvany, G.C. Sharp.
Immunogenetic studies of juvenile dermatomyositis: III. Study of antibody to organ-specific and nuclear antigens.
Arthritis Rheum, 28 (1985), pp. 151-157
[12.]
S.L. Bowyer, R.A.F. Clark, C.G. Ragsdale, J.R. Hollister, D.B. Sullivan.
Juvenile dermatomyositis: histological findings and pathogenetic hypothesis for the associated skin changes.
J Rheumatol, 13 (1986), pp. 753-759
[13.]
A.A. Amato, R.J. Barohn.
Idiopathic inflammatory myopathies.
Neurol Clin, 15 (1997), pp. 615-644
[14.]
M.C. Dalakas.
Polymiositis, dermatomyiositis, and inclusion body-myositis.
N Engl J Med, 325 (1991), pp. 1487-1498
[15.]
N.E. Bowles, V. Dubowitz, C.A. Sewry, L.C. Archard.
Dermatomyositis, polymyositis, and coxsackie-B-virus infection.
Lancet, 1 (1987), pp. 1004-1007
[16.]
F. Lapetina.
Toxoplasmosis and dermatomyositis: a causal orcasual relationship.
Pediatr Med Chir, 11 (1989), pp. 197-203
[17.]
R.A. Pittsley, M.A. Shearn, M.D. Kaufman.
Acute hepatitis B simulating dermatomyositis.
Jama, 239 (1978), pp. 959
[18.]
R.M. Lewkonia, D. Horne, M.R. Dawood.
Juvenile dermatomyositis in a child infected with human parvovirus B19.
Clin InfectDis, 21 (1995), pp. 430-432
[19.]
T.R. Woo, J. Rasmussen, J.P. Callen.
Recurrent photosensitive dermatitis preceding juvenile dermatomyositis.
Pediatr Dermatol, 2 (1985), pp. 207-212
[20.]
J.T. Kissel, J.R. Mendell, K.W. Rammohan.
Microvascular deposition of complement membrane attack complex in dermatomyositis.
N Engl J Med, 314 (1986), pp. 329-334
[21.]
L.G. Rider, S. Okada, D.D. Sherry, C.A. Wallace, L.S. Zemel, J.C. Jacobs.
Epidemiologic features and environmental exposures associated with illness onset in juvenile inflammatory myopathy.
Arthritis Rheum, 38 (1995), pp. 362
[22.]
B. Sigurgeirsson, B. Lindelöf, O. Edhag, E. Allander.
Risk of cancer in patients with dermatomyositis or polymyositis. A population-based study.
N Engl J Med, 326 (1992), pp. 363-367
[23.]
M.C. Dalakas.
Clinical, immunopathologic, and therapeutic considerations of inflammatory myopathies.
Clin Neuropharmacology, 15 (1992), pp. 327-351
[24.]
L.M. Pachman.
An update on juvenile dermatomyositis.
CurrOpin Rheumatol, 7 (1995), pp. 437-441
[25.]
P.N. Malleson.
Controversies in Juvenile Dermatomyositis.
J Rheumatol, 22 (1990), pp. 1-6
[26.]
B.M. Feldman, M. Reichlin, R.M. Laxer, I.N. Targoff, L.D. Stein, E.D. Silverman.
Clinical significance of specific autoantibodies in juvenile dermatomyositis.
J Rheumatol, 23 (1996), pp. 1794-1797
[27.]
J.P. Scott, C. Arroyave.
Activation of complement and coagulationin juvenile dermatomyositis.
Arthritis Rheum, 30 (1987), pp. 572-576
[28.]
S.L. Bowyer, C.E. Blane, D.B. Sullivan.
Childhood dermatomyositis: factors predicting functional outcome and development of dystrophic calcification.
J Pediatr, 103 (1983), pp. 888
[29.]
M.A. Dawkins, J.L. Jorizzo, F.O. Walker, D. Albertson.
Dermatomyositis: a dermatology-based case series.
J Am Acad Dermatol, 38 (1998), pp. 397-404
[30.]
M.A. Pachman, A.M. Callen, J.R. Hayford, A. Chung, J. Sinacore, R. Ramsey-Goldman.
Juvenile dermatomyositis (JDMS): decreasedcalcinosis (Ca++) with intermittent high-dose intravenous methylprednisolone (iv pulse) therapy.
Arthritis Rheum, 37 (1994), pp. 429
[31.]
A. Cosnes, F. Amaudric, R. Gheraldi, J. Verroust, J. Wechsler, J. Revuz.
Dermatomyositis without muscle weakness. Longterm follow-up of 12 patients without systemic corticosteroids.
Arch Dermatol, 131 (1995), pp. 1381-1385
[32.]
N.R. Rockerbie, T.Y. Woo, J.P. Callen, T. Giustina.
Cutaneouschanges of dermatomyositis precede muscle weakness.
J AmAcad Dermatol, 20 (1989), pp. 629-632
[33.]
T.J.A. Lehman.
Aggressive therapy for childhood rheumatic diseases. When are immunosuppresives appropriate?.
Arthritis Rheum, 36 (1993), pp. 71-74
[34.]
L.C. Miller, B.A. Sisson, L.B. Tucker, B.A. DeNardo, J.G. Schaller.
Methotrexate treatment of recalcitrant childhood dermatomyositis.
Arthritis Rheum, 35 (1992), pp. 1143-1149
[35.]
A. Sansome, V. Dubowitz.
Intravenous immunoglobulin in juvenile dermatomyositis-four year review of nine cases.
Arch Dis Child, 72 (1995), pp. 25-28
[36.]
E. Collet, S. Dalac, B. Maerens, J.M. Courtois, M. Izac, D. Lambert.
Juvenile dermatomyositis: treatment with intravenous gamma globulin.
Br J Dermatol, 130 (1994), pp. 231-234
[37.]
M. Borrelli, M.J. Prado, P. Cordeiro, E.R. Wroclawski, J.R. Monteino, N.H. kiss.
Ureteral necrosis in dermatomyositis.
J Urol, 139 (1988), pp. 1275-1277
[38.]
G. Citera, G. Espada, J.A. Maldonado Cocco.
Sequential of two connective tissue diseases in juvenile patients.
J Rheumatol, 20 (1993), pp. 2149-2152
Copyright © 2000. Asociación Española de Pediatría
Idiomas
Anales de Pediatría

Suscríbase a la newsletter

Opciones de artículo
Herramientas
es en

¿Es usted profesional sanitario apto para prescribir o dispensar medicamentos?

Are you a health professional able to prescribe or dispense drugs?