Upper eyelid juvenile xanthogranuloma: a case report

ABSTRACT Juvenile xanthogranuloma is a rare benign non-Langerhans cell histiocytosis. Clinical manifestation usually occurs up to the age of 2 years, with yellowish papules and variable clinical progression. Approximately 0.75% of patients had systemic involvement and 0.25%, ocular alterations. The purpose of this report is to describe a case of a preschool 2-year-old female patient, with nodules in the upper right eyelid, 0.5-cm wide, with well-defined edges, an uncertain date of onset, a stable growth for 6 months, with no inflammatory signs, pruritus, pain, bleeding, or other similar lesions in the body. No further changes were observed in the physical examination. Histopathological examination of the specimen showed a skin lesion with histiocytoid, spindle-shaped cells and xanthomized cells, inflammatory infiltrate and numerous Touton giant cells. The result was compatible with diagnosis of juvenile xanthogranuloma. Therefore, the importance of including juvenile xanthogranuloma in the differential diagnosis of eyelid lesions is emphasized, especially in children.【摘要】幼年黄色肉芽肿（Juvenile xanthogranuloma）是一种罕见的良性非朗格汉斯细胞组织细胞增生症（non-Langerhans cell histiocytosis）。该病临床表现多始发于2岁以内，以淡黄色丘疹为典型表现，临床病程存在显著异质性。约0.75%的患者可出现系统受累，另有0.25%的患者存在眼部病变。本病例报告旨在介绍1例2岁学龄前女性患儿：患儿右侧上眼睑可见直径0.5cm的结节，边界清晰，发病时间不明，近6个月来结节生长稳定，无炎性体征、瘙痒、疼痛、出血表现，全身其余部位未发现类似皮损，体格检查未见其他异常。对手术标本进行组织病理学检查可见皮损区域存在组织细胞样梭形细胞、黄瘤样细胞、炎性浸润灶及大量杜顿巨细胞（Touton giant cells），该结果符合幼年黄色肉芽肿的诊断标准。因此，临床需重视将幼年黄色肉芽肿纳入儿童眼睑病变的鉴别诊断范畴。