Actas Dermo-Sifiliográficas (English Edition) Actas Dermo-Sifiliográficas (English Edition)
Actas Dermosifiliogr 2018;109:312-22 - Vol. 109 Num.4 DOI: 10.1016/j.adengl.2018.03.011
Review
Morphea in Childhood: An Update
Morfea en la infancia: actualización
B. Aranegui, , J. Jiménez-Reyes
Servicio de Dermatología, Hospital Universitario Infanta Cristina , Parla, Madrid, Spain
Received 22 January 2017, Accepted 25 June 2017
Abstract

Morphea is an inflammatory, fibrosing skin disorder. When it occurs in childhood, it is also known as juvenile localized scleroderma. It is more common in girls and typically appears around the age of 5 to 7 years. According to a recent classification system, morphea is divided into 5 types: circumscribed (plaque), linear, generalized, pansclerotic, and mixed. Approximately 40% of patients present extracutaneous manifestations. Childhood morphea is treated with phototherapy, oral or topical calcitriol, topical tacrolimus 0.1%, methotrexate, topical or systemic corticosteroids, mycophenolate mofetil, bosentán, and topical imiquimod 5%. A variety of measuring tools are used to monitor response to treatment. Few prognostic studies have been conducted, but findings to date suggest that the disease tends to run a chronic or intermittent-recurrent course and frequently causes sequelae.

Resumen

La morfea es una enfermedad de la piel que se manifiesta en forma de inflamación y fibrosis. En niños y jóvenes, también se conoce como esclerodermia juvenil localizada. En edad infantil, afecta con mayor frecuencia al sexo femenino y la edad de comienzo se ha establecido en torno a los 5-7 años. Una clasificación reciente divide la morfea en: circunscrita (en placas), lineal, generalizada, panesclerótica y mixta. Alrededor de un 40% de los pacientes presentan manifestaciones extracutáneas.

Los tratamientos empleados en morfea infantil son: fototerapia, calcitriol oral, calcipotriol tópico, tacrolimus 0,1% tópico, metotrexato, glucocorticoides tópicos y sistémicos, mofetil micofenolato, bosentán e imiquimod 5% tópico. Diversas medidas de resultado pueden ayudar a monitorizar el tratamiento. Los estudios pronósticos son escasos, pero apuntan hacia una enfermedad con tendencia a un curso crónico o intermitente-recurrente y una frecuencia considerable de secuelas.

Keywords
Morphea, Childhood, Localized juvenile scleroderma, Linear scleroderma, Facial hemiatrophy, Review literature as topic, Phototherapy, Methotrexate
Palabras clave
Morfea, Infancia, Esclerodermia juvenil localizada, Esclerodermia lineal, Hemiatrofia facial, Revisión narrativa, Fototerapia, Metotrexato
Actas Dermosifiliogr 2018;109:312-22 - Vol. 109 Num.4 DOI: 10.1016/j.adengl.2018.03.011
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