Ramos Suárez A, Ayet Roger I, Serra Castanera A
Servicio de Oftalmología, Hospital Sant Joan de Déu, Esplugues de Llobregat, Barcelona, España; Departamento de Oftalmología, Hospital Costa del Sol, Marbella, Málaga, España.
Servicio de Oftalmología, Hospital Sant Joan de Déu, Esplugues de Llobregat, Barcelona, España.
Arch Soc Esp Oftalmol. 2017 Aug;92(8):394-397. doi: 10.1016/j.oftal.2017.02.012. Epub 2017 May 15.
The case concerns a 22 day-old male child with juvenile xanthogranuloma (JXG), which manifested as a unilateral glaucoma and with fibrinous haemorrhagic exudate in the anterior chamber affecting the angle of the right eye. Despite a high level of suspicion, the definitive diagnosis was not possible until the infant reached the age of 10 months, after the appearance of the skin lesions typical of this condition and histopathological study of them.
JXG is a rare disease, characterised by yellowish skin lesions on the trunk, neck, or head. Up to 10% of cases will have ocular involvement, which is the most common extracutaneous manifestation of the disease.
该病例为一名22日龄男童,患有幼年性黄色肉芽肿(JXG),表现为单侧青光眼,前房有纤维蛋白性出血性渗出物,累及右眼房角。尽管高度怀疑,但在婴儿出现该疾病典型的皮肤病变并进行组织病理学研究后,直到10个月大才得以确诊。
JXG是一种罕见疾病,特征为躯干、颈部或头部出现淡黄色皮肤病变。高达10%的病例会有眼部受累,这是该疾病最常见的皮肤外表现。