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Vol. 109. Num. 6.July - August 2018
Pages e37-e46Pages 465-576
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Vol. 109. Num. 6.July - August 2018
Pages e37-e46Pages 465-576
e- Case Report
DOI: 10.1016/j.adengl.2018.05.014
Juvenile Dermatomyositis: Key Roles of Muscle Magnetic Resonance Imaging and Early Aggressive Treatment
La resonancia magnética muscular y el tratamiento agresivo precoz, claves en la dermatomiositis juvenil
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O. Corral-Magañaa,
Corresponding author
oricmag@gmail.com

Corresponding author.
, A.F. Bauzá-Alonsoa, M.M. Escudero-Góngoraa, L. Lacruzb, A. Martín-Santiagoa
a Servicio de Dermatología, Hospital Universitario Son Espases, Palma de Mallorca, España
b Servicio de Pediatría, Hospital Universitario Son Espases, Palma de Mallorca, España
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Tables (3)
Table 1. Clinical Characteristics of Patients.
Table 2. Initial Treatment.
Table 3. Maintenance Treatment.
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Abstract

Juvenile dermatomyositis is a rare systemic connective tissue disease with onset during childhood. It presents clinically with proximal muscle weakness and characteristic skin involvement. Diagnosis is based on the Bohan and Peter criteria, though many authors are now substituting biopsy with muscle magnetic resonance imaging (MRI) for both diagnosis and follow-up. Without intensive early treatment, complications such as calcinosis cutis and lipodystrophy can develop in the chronic phases of the disease. Early recognition is therefore key to management. We present a series of 5 patients who were diagnosed with Juvenile dermatomyositis on muscle MRI without undergoing muscle biopsy and who received early treatment. We draw attention to the usefulness of muscle MRI for the diagnosis of muscle involvement and to the importance of early initiation of intensive treatment to prevent complications.

Keywords:
Juvenile dermatomyositis
Muscle magnetic resonance
Early treatment
Resumen

La dermatomiositis juvenil es una conectivopatía sistémica infrecuente de aparición en la edad pediátrica. Clínicamente se caracteriza por la presencia de debilidad muscular proximal con afectación cutánea característica. El diagnóstico de la enfermedad se realiza mediante los criterios de Bohan y Peter, si bien recientemente numerosos autores están sustituyendo la realización de biopsia muscular por la resonancia magnética muscular (RMM) para el diagnóstico y seguimiento de la enfermedad.

En fases crónicas de la enfermedad, y sin un tratamiento precoz intensivo, pueden aparecer complicaciones como la calcinosis cutánea o la lipoatrofia, por lo que el reconocimiento temprano de la enfermedad es clave en el manejo.

Presentamos una serie de 5 pacientes diagnosticados de dermatomiositis juvenil mediante RMM sin realizarse biopsia muscular y tratados de forma temprana. Resaltamos la utilidad de la RMM en el diagnóstico de la enfermedad muscular y la importancia de instaurar el tratamiento de forma precoz e intensiva para prevenir las complicaciones.

Palabras clave:
Dermatomiositis juvenil
Resonancia magnética muscular
Tratamiento precoz

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