ABSTRACT
Resumen: Introducción: La esclerodermia localizada es la forma de presentación de esclerosis más frecuente en niños; tiene una incidencia de 2.7 por cada 100,000 habitantes, con predilección por la raza caucásica y el sexo femenino con relación de 2.4 a 4.1 por cada varón. El común denominador es la afección a nivel de tejido conectivo, genera una variedad de presentación clínica que va desde placas escleróticas localizadas circunscritas que afectan la piel hasta afecciones más profundas que atacan al tejido muscular y óseo, dejando secuelas estéticas y/o deformidades incapacitantes en el paciente. Objetivo: Presentación de caso y su manejo quirúrgico. Los datos expuestos se obtuvieron del expediente clínico físico y electrónico, entrevista directa con paciente y familiares y seguimiento y valoración de estudios radiográficos, desde Enero de 2012 hasta Noviembre de 2017. Discusión: El tratamiento quirúrgico está indicado cuando las deformidades y contracturas condicionan incapacidad en el paciente. Los mejores resultados se obtendrán una vez que la enfermedad haya dejado de progresar.
Abstract: Introduction: Localized scleroderma is the most common form of sclerosis in children; it has an incidence of 2.7 per 100,000 inhabitants, with a predilection for the Caucasian and female races of 2.4 to 4.1 per male. The common denominator is the connective tissue-level condition, causing a variety of clinical presentation ranging from localized sclerotic circumscribed plaques affecting the skin, to deeper conditions that attack muscle and bone tissue, leaving aesthetic consequences and/or disabling deformities in the patient. Objective: Case presentation and surgical management. The data presented were obtained from the physical and electronic clinical record, direct interview with patients and family members and monitoring and evaluation of radiographic studies, from January 2012 to November 2017. Discussion: Surgical treatment is indicated when deformities and contractures condition incapacity in the patient. The best results will be obtained once the disease has stopped progressing.
Subject(s)
Humans , Male , Female , Child , Scleroderma, Localized/surgery , Scleroderma, Localized/diagnosis , Orthopedic Procedures/methods , Physical Examination , Disease ProgressionABSTRACT
A large number of diseases may cause Atrophic skin disorders are caused by a large number of diseases, some of them idiopathic and others inflammatory, in which there is loss of volume of body segments. Localized scleroderma is a rare inflammatory dermatosis, manifested by atrophic skin and subcutaneous tissue alterations. Lipoatrophy may be genetically inherited or acquired as a result of panniculitis, HIV infections or aging. Many treatments have been proposed. Results vary in the acute inflammatory phase and are scarce when sclerosis and atrophy have already been established. This article describes four cases of localized facial scleroderma and one of facial idiopathic lipoatrophy treated with implantation of autologous fat globules extracted from the infragluteal groove, without utilization of cannula aspiration, with lasting results.
Os distúrbios atróficos da pele abrangem inúmeras doenças, algumas idiopáticas e outras, inflamatórias, em que há perda do volume de segmentos do corpo. A esclerodermia localizada é uma dermatose inflamatória, rara, que pode manifestar-se com alterações atróficas da pele e tecido subcutâneo. A lipoatrofia pode ser herdada geneticamente ou adquirida relacionada a paniculites, infeccção pelo HIV, ou envelhecimento. Muitos tratamentos são propostos. Os resultados são variáveis na fase inflamatória aguda e apresentam pouca resposta quando a esclerose e atrofia já estão instaladas. Descreve-se o tratamento de quatro casos de esclerodermia localizada e um de lipoatrofia idiopática, na face, tratados com enxerto de fragmentos de lóbulos de gordura autóloga extraídos sem a utilização da cânula aspirativa, com resultados duradouros.