Xantogranuloma juvenil: experiencia en un hospital pediátrico

Introduccion. El xantogranuloma juvenil (XGJ) es una histiocitosis de celulas no Langerhans, de curso benigno y autolimitado, que afecta principalmente a lactantes y ninos. Los pacientes pueden presentar compromiso extracutaneo, y las localizaciones mas frecuentes son ojos, higado, pulmon y SNC. Objetivos. 1) Conocer las caracteristicas clinicas de los pacientes con XGJ evaluados en el Servicio de Dermatologia del Hospital de Pediatria “Prof. Dr. Juan P. Garrahan”. 2) Describir las enfermedades asociadas y las posibles complicaciones. 3) Conocer la evolucion de los pacientes. Materiales y metodos. Se realizo un trabajo retrospectivo, observacional y longitudinal, en el que se estudiaron todos los pacientes con diagnostico clinico e histopatologico de XGJ desde agosto de 1988 hasta diciembre de 2006. Resultados. Presentaron diagnostico clinico de XGJ 86 pacientes, se obtuvo la confirmacion histologica en 45 de ellos y se excluyo a los 41 pacientes restantes. En el 67% la enfermedad comenzo dentro del primer ano de vida. En el 44% las lesiones eran unicas y en el 56% multiples. La localizacion mas frecuente fue el tronco 41%, seguido por la extremidad cefalica 33% y miembros 26%. El compromiso extracutaneo se presento clinicamente en 4 pacientes (9%): hipema y glaucoma en 3, y compromiso multisistemico en 1. Conclusion. El XGJ es una enfermedad de curso benigno y autorresolutivo, limitada en la mayoria de los casos a la piel, y no requiere tratamiento alguno. Sin embargo debe realizarse un seguimiento clinico multidisciplinario de los pacientes, con el fi n de identifi car compromiso extracutaneo y sus posibles complicaciones (Dermatol Argent 2010;16(4):262-267). Palabras clave: xantogranuloma juvenil, histiocitosis de celulas no Langerhans,infancia. Title: Juvenile xanthogranuloma: experience in a children’s hospital Abstract Background . Juvenile Xanthogranuloma (JXG), a non Langerhans histiocytosis, is a benign, self-healing disorder that affects mainly children. Extracutaneous involvement may also be present, such as ocular, liver, lung o central nervous system. Objectives. 1- To identify the clinical features of patients with diagnosis of JXG as seen in the Department of Dermatology, Hospital de Pediatria “Prof. Dr. Juan P. Garrahan”. 2- To describe the associated diseases and the complications of these patients. 3- To describe their outcome. Materials and methods. Retrospective, observational, and longitudinal study which included all the patients with clinical and histopathological features of JXG evaluated since August 1998 to December 2006 in our Department. Results. Clinical diagnosis of JXG was made in 86 patients and confirmed by biopsy in 45 of them. 41 patients were excluded because they didn't have histopathological confirmation. In 67% of the patients the disease appeared during the first year of life. The lesions were solitary (44%) or multiple (56%), and the most common location was the trunk (41%), followed by the head (33%) and extremities (26%). Extracutaneous involvement was present in 4 patients (9%), 3 of them with ocular manifestation with hyphema and glaucoma, and one presented mulitisystemic involvement. Conclusions. JXG is a benign, self-healing disorder, which in the vast majority of cases is limited to the skin and requires no treatment. Nevertheless, an adequate multidisciplinary follow-up must be done to identify the extracutaneous involvement and its complications (Dermatol Argent 2010;16(4):262-267). Key words: juvenile xanthogranuloma, non Langerhans histiocytosis, children.