Castro Patrícia Aparecida de, Urbano Lílian Mendes Ferreira, Costa Izelda Maria Carvalho
Departamento de Dermatologia, Universidade de Brasília, DF, Brasil.
An Bras Dermatol. 2009 Jul-Aug;84(4):317-29. doi: 10.1590/s0365-05962009000400002.
Kawasaki disease is a systemic acute vasculitis of unknown etiology. It is the leading cause of acquired heart disease in children in the USA. It occurs more frequently in boys and eighty percent of the cases occur in children under five years of age. The disease rarely occurs after eight years and it can affect children of all races, with higher incidence among Asian descendants. Kawasaki disease is characterized by fever, bilateral non-exudative conjunctivitis, redness and swelling of the tongue, lips and oral mucosa, abnormalities in the extremities, cervical lymph node, and polymorphic exanthema. Aneurysms and stenoses of coronary arteries occur in approximately 20 to 25% of untreated patients and subsequently can lead to acute myocardial infarction and sudden death. Treatment with intravenous immunoglobulin is effective and should be initiated early to prevent cardiac sequel. The development of diagnostic tests, more specific treatment approaches and prevention of this potentially fatal disease in children depends on continuous advances in the determination of its pathogenesis.
川崎病是一种病因不明的全身性急性血管炎。它是美国儿童后天性心脏病的主要病因。该病在男孩中更为常见,80%的病例发生在5岁以下儿童。该疾病很少在8岁后出现,可影响所有种族的儿童,在亚洲后裔中发病率较高。川崎病的特征为发热、双侧非渗出性结膜炎、舌头、嘴唇和口腔黏膜红肿、四肢异常、颈部淋巴结肿大以及多形性皮疹。未经治疗的患者中约20%至25%会出现冠状动脉瘤和狭窄,随后可能导致急性心肌梗死和猝死。静脉注射免疫球蛋白治疗有效,应尽早开始以预防心脏后遗症。诊断测试的发展、更具特异性的治疗方法以及预防儿童这种潜在致命疾病,都依赖于其发病机制研究的不断进展。