Gupta Aman, Singh Surjit
Allergy Immunology Unit, Department of Pediatrics, PGIMER, Chandigarh, India.
Indian Dermatol Online J. 2016 Nov-Dec;7(6):461-470. doi: 10.4103/2229-5178.193903.
Kawasaki disease (KD) is a systemic vasculitis that mostly affects children below the age of 5. The vasculitis involves arteries of medium size, especially the coronaries. Various etiologies have been proposed including association with micro-organisms, bacterial superantigens, and genetic factors, however, the exact cause remains unknown. There is no specific laboratory test for KD. Diagnosis is clinical and depends upon the presence of fever for ≥5 days and 4 or more of five principal features, viz. polymorphous exanthem, extremity changes, mucosal changes involving the lips and oral cavity, bilateral bulbar conjunctival injection, and unilateral cervical lymphadenopathy. The term "incomplete KD" refers to the presence of fever and less than four principal clinical features. Recognition of this group of patients is important because it is usually seen in infants and risk of coronary abnormalities is increased probably because of delays in diagnosis. However, what appears to be "incomplete" at a given point of time may not actually be so because some of the features may have already subsided and others may evolve over time. Hence, a detailed dermatological examination is warranted in all cases, especially in incomplete KD, to ensure timely diagnosis. Although KD is a self-limiting disease in most patients, coronary artery abnormalities (CAAs) including coronary dilatations and aneurysms may develop in up to 25% of untreated patients. CAAs are the most common cause of morbidity and mortality in patients with KD. Treatment is aimed at reducing inflammation and consists of intravenous immunoglobulin (IVIG) along with aspirin. Despite treatment, some patients may still develop CAAs, and hence, long-term follow up is of utmost importance.
川崎病（KD）是一种主要影响5岁以下儿童的全身性血管炎。这种血管炎累及中等大小的动脉，尤其是冠状动脉。已经提出了各种病因，包括与微生物、细菌超抗原和遗传因素的关联，然而，确切病因仍然未知。目前尚无针对川崎病的特异性实验室检查。诊断依靠临床症状，取决于发热≥5天以及具备五项主要特征中的4项或更多，即多形性皮疹、四肢变化、涉及嘴唇和口腔的黏膜变化、双侧球结膜充血以及单侧颈部淋巴结肿大。“不完全川崎病”一词指的是存在发热但主要临床特征少于4项。识别这组患者很重要，因为这类情况通常见于婴儿，可能由于诊断延迟导致冠状动脉异常的风险增加。然而，在某个特定时间点看似“不完全”的情况实际上可能并非如此，因为一些特征可能已经消退，而其他特征可能会随时间演变。因此，所有病例，尤其是不完全川崎病，都需要进行详细的皮肤科检查，以确保及时诊断。尽管川崎病在大多数患者中是一种自限性疾病，但在未经治疗的患者中，高达25%可能会出现包括冠状动脉扩张和动脉瘤在内的冠状动脉异常（CAA）。冠状动脉异常是川崎病患者发病和死亡的最常见原因。治疗旨在减轻炎症，包括静脉注射免疫球蛋白（IVIG）和阿司匹林。尽管进行了治疗，一些患者仍可能出现冠状动脉异常，因此，长期随访至关重要。
