Wisuthsarewong W, Viravan S, Manonukul J
Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand.
J Med Assoc Thai. 2000 Nov;83(11):1415-9.
Sarcoidosis is a multisystemic granulomatous disorder of unknown etiology, most commonly affecting young adults and presenting most frequently with bilateral hilar lymphadenopathy, pulmonary infiltration, and skin or eye lesions. Sarcoidosis in children under the age of 4, preschool sarcoidosis, is rare and has very different clinical mainfestations characterized by the typical triad of skin, eye and joint involvement without lung disease. Diagnosis is established when compatible clinical findings are supported by histological evidence of widespread noncaseating epithelioid-cell granulomas in more than one organ or a positive Kveim-Siltzbach skin test. This report describes a 2-year-old Thai girl with typical clinical manifestations of preschool sarcoidosis. The histopathological studies of tissue specimens and the laboratory investigations support the diagnosis of sarcoidosis. The patient responded to systemic corticosteroid therapy.
结节病是一种病因不明的多系统肉芽肿性疾病,最常累及年轻人,最常见的表现为双侧肺门淋巴结肿大、肺部浸润以及皮肤或眼部病变。4岁以下儿童的结节病,即学龄前结节病,较为罕见,其临床主要表现差异很大,特征为典型的皮肤、眼睛和关节受累三联征,而无肺部疾病。当多个器官广泛的非干酪样上皮样细胞肉芽肿的组织学证据或阳性的Kveim-Siltzbach皮肤试验支持相符的临床发现时,即可确立诊断。本报告描述了一名患有学龄前结节病典型临床表现的2岁泰国女孩。组织标本的组织病理学研究和实验室检查支持结节病的诊断。该患者对全身皮质类固醇治疗有反应。