Symmetrical Giant Facial Plaque-Type Juvenile Xanthogranuloma; A New Case Report

摘要

Juvenile xanthogranuloma is the most common non-Langerhans histiocytosis (non-LCH). The vast majority of lesions are diagnosed within the first year of life and about 80% of cases are solitary. Symmetrical giant facial plaque-type juvenile xanthogranuloma (SGFP-JXG) is a rare and new variant of juvenile xanthogranuloma (JXG), reported only in three cases in the medical literature and it was originally reported by GunsonandBirchall in 2008. Herein we report a new case of SGFP-JXG as a thirty months old girl, presented with seven months history of a diffuse asymptomatic yellowish to orange butterfly plague eruption on the face, and papular eruption on the trunk and upper limbs. A skin biopsy with immunohistochemistry showed a Non-langerhan histiocytosis type juvenile xanthogranuloma. We emphasise the importance of knowing this rare variant with atypical presentation that makes the diagnosis of JXG extremely challenging.