Introducción. “Esclerodermia” significa “piel dura”. El término. “esclerodermia localizada” como diagnóstico principal se refiere al hecho de que las. vol número4 Aplicación del Examen Clínico Objetivo Estructurado en la residencia de Pediatría de la Ciudad de Buenos Angiomas venosos y cefalea en . Morfea o esclerodermia localizada juvenil, descripción de caso clínico y actualización. 9. Arkachaisri T, Vilaiyuk S, Li S, O’Neill KM, Pope E, Higgins.
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Morfea o esclerodermia localizada juvenil: caso clínico
In juvenile localized scleroderma JLSnew techniques such as Doppler and laser Doppler imaging have shown their usefulness for the daily monitoring of the patients.
In juvenile systemic sclerosis JSSca new severity eesclerodermia has been developed and needs to be validated in future trials.
Morphea or juvenile localised scleroderma: Recent studies, concerning histopathology and clinical associations with other conditions, open new horizons on the etiopathogenesis of scleroderma. Studies over recent years highlighted the role of imaging as outcome measures for JLS and introduced a severity score for JSSc. Morphea or juvenile localised scleroderma JLS is an autoimmune, inflammatory, chronic, slowly progressive connective tissue disease of unknown cause that preferably affects skin and underlying tissues.
The case is presented of an 8 year-old girl who presented with pediatia hypopigmented plaques, of linear distribution in the right upper extremity of two years onset, together with papery texture hyperpigmented indurated plaques with whitish areas of thinned skin in right lower extremity, and leg and ankle swelling.
The clinical features and diagnostic tests, including histology were compatible with linear and pansclerotic JLS.
She started with immunosuppressive therapy, physiotherapy, and occupational therapy. Finally, a randomized, double-blind controlled trial, a multicenter consensus statement and long-term follow-up studies have confirmed the important role of methotrexate MTX for the treatment of JLS.
New developments have been also reached in the field of outcome measures. Early diagnosis, appropriate assessment and effective treatment are crucial to improve the long-term outcome. Scleroderma, in its localized and systemic presentation, represents the third most frequent rheumatic condition in childhood after juvenile idiopathic arthritis and systemic pediwtria erythematosus.
Morphea; Juvenile localised scleroderma; Autoimmune. To report a case of Juvenil Localised scleroderma in an 8-year old girl, contributing to an early diagnosis and treatment.
We report a case of linear and pansclerotic ELJ type, in which there was a 2 year delay in diagnosis, however the response to treatment was positive as expected.