A Review of 10 Cases with Solitary Cutaneous Juvenile Xanthogranuloma-Clinical and Histological Presentation

Abstract

Juvenile xanthogranuloma (JXG) is one of the most common forms of non-Langerhans' cell histiocytosis in infancy and early childhood. It is presented principally as a solitary cutaneous lesion with predilection for the head, neck and upper trunk region. Cutaneous lesions usually resolve and most patients have an otherwise unremarkable course. Systemic (extracutaneous) involvement is rare but significant morbidity and occasional deaths may occur. A rare association between juvenile xanthogranuloma and neurofibromatosis Ⅰ or juvenile chronic myelogenous leukemia has been identified. This study included 10 patients with a solitary cutaneous form of juvenile xanthogranuloma at our institute in past 20 years. The patient age, gender, lesion size, location, associated diseases, clinical, management, follow-up and histiologic characteristic are summarized and the literatures are reviewed.