JUVENILE XANTHOGRANULOMA: A CASE REPORT

ABSTRACT Objective: To report a rate case of Juvenile xanthogranuloma in a newborn infant. Case description: We present the case of a 31-week preterm newborn with multiple skin lesions whose clinical, histological and immunohistochemical findings allowed the diagnosis of juvenile xanthogranuloma. Currently, the patient has nine months-old, and there is no aggravation of the skin lesions or evidence of extra-cutaneous involvement, particularly ophthalmic. Comments: Juvenile xanthogranuloma is a rare and benign condition, included in the vast group of non-Langerhans histiocytosis. It typically occurs in the pediatric age and may have a neonatal presentation. It affects predominantly the skin, in the form of papules or yellow and/or erythematous nodules and could be asymptomatic, multiple or solitary. Extra-cutaneous involvement, is more common in toddlers and when multiple lesions are present. The eye is the most affected site. We highlight this clinical case by its presentation in the neonatal period and in the form of multiple lesions, which bestows an increased risk of extra-cutaneous involvement, although this has not yet been verified.

摘要
目的：本文旨在报告1例新生儿幼年性黄色肉芽肿（Juvenile xanthogranuloma）的罕见病例。
病例介绍：本文报告1例孕31周早产新生儿，该患儿出现多发性皮肤病变，结合临床特征、组织病理学及免疫组织化学检查结果，确诊为幼年性黄色肉芽肿。目前患儿已9月龄，皮肤病变未见加重，亦未出现皮肤外受累证据，尤其未检出眼部受累情况。
讨论：幼年性黄色肉芽肿属于非朗格汉斯细胞组织细胞增生症（non-Langerhans histiocytosis）大类中的罕见良性病变，通常好发于儿童时期，亦可出现新生儿期发病的情况。该病主要累及皮肤，表现为丘疹或黄色及/或红斑性结节，可无自觉症状，皮损可为多发或单发。皮肤外受累更多见于幼儿期，且常伴随多发皮损，眼部是最常受累的部位。本例患儿于新生儿期发病且皮损多发，该情况理论上会增加皮肤外受累的风险，但目前该风险尚未得到证实，特此报告此病例。