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Abstract：
Juvenile xanthogranuloma (JXG) is the most common form of non-Langerhans cell histiocytosis in childhood. It often presents with cutaneous involvement and exhibits a predilection for the head and neck region. This article illustrates a case of congenital JXG in a 5-month-old boy, characterized by a solitary, well-circumscribed nodule above the left upper lip. Histopathologically, the lesion exhibited histiocytes with eosinophilic cytoplasm and Touton giant cells. Immunohistochemistry revealed histiocytes positive for CD68 and Factor XIIIa, while negative for S-100 protein. Clinicians should become familiar with the broad clinical spectrum of cutaneous JXG, particularly its congenital presentation, in order to ensure timely and accurate management.
摘要：
幼年黄色肉芽肿（JXG）是儿童期最常见的非朗格汉斯细胞组织细胞增生症。它通常表现为皮肤受累，并表现出对头部和颈部区域的偏爱。本文说明了一个5个月大男孩的先天性JXG病例，以孤独为特征，在左上唇以上的界限清楚的结节。组织病理学,病变呈组织细胞伴嗜酸性细胞浆和Touton巨细胞。免疫组织化学显示CD68和因子XIIIa的组织细胞呈阳性，而S-100蛋白阴性。临床医生应该熟悉皮肤JXG的广泛临床谱，特别是它的先天性表现，以确保管理及时准确。
