Post Graduate Institute of Medicine, Colombo, Sri Lanka.
Department of Paediatrics, Faculty of Medicine, University of Kelaniya, Ragama, Sri Lanka.
BMC Pediatr. 2022 Jul 23;22(1):444. doi: 10.1186/s12887-022-03510-z.
Kawasaki disease (KD) is a medium and small vessel vasculitis which usually has a good response to immunoglobulin therapy (IVIG). We present a case of incomplete KD with IVIG resistance associated with an unusual combination of vesicular guttate-psoriasiform rash, hypertension and late onset small joint arthritis.
A four-month-old male infant from Sri Lanka presented with high fever, conjunctival redness, pedal oedema and skin rash. He was found to have hypertension since admission with a high white cell count and high inflammatory markers. There was poor response to intravenous antibiotics and subsequent 2D echocardiogram revealed coronary artery aneurysms suggestive of KD. In the third week of illness he developed a vesiculo-papular rash involving face, trunk and limbs - which on biopsy revealed features of guttate psoriasis. Fever spikes continued and the coronary arteries showed progressive dilatation despite timely intravenous immunoglobulin administered on day 6 and methylprednisolone administered on day 10-13. Therapeutic response by means of reduction of fever was seen only after initiation of intravenous infliximab on day 28 of illness for which the fever responded within 24 hours. He developed a small joint arthritis of hands and feet on day 40 of illness which responded only after initiating methotrexate therapy. The hypertension persisted for 4 months after the onset of the illness before complete resolution.
This case report depicts an unusual presentation of KD with a vesicular guttate-psoriasiform eruption, hypertension and late onset small joint arthritis. It highlights that clinicians should be aware of the fact that KD could present with such atypical manifestations and could develop unusual complications.
川崎病（KD）是一种中小血管血管炎，通常对免疫球蛋白治疗（IVIG）有良好的反应。我们报告了一例不完全性 KD 伴 IVIG 耐药，伴有不寻常的水疱状斑片状银屑病样皮疹、高血压和迟发性小关节关节炎的组合。
一名来自斯里兰卡的四个月大男婴出现高热、结膜充血、足肿和皮疹。入院时发现他有高血压，白细胞计数和炎症标志物升高。静脉注射抗生素反应不佳，随后的 2D 超声心动图显示冠状动脉动脉瘤，提示 KD。在疾病的第三周，他出现了涉及面部、躯干和四肢的水疱状丘疹样皮疹 - 活检显示为点滴状银屑病的特征。发热持续升高，尽管在第 6 天和第 10-13 天给予静脉免疫球蛋白和甲基强的松龙治疗，但冠状动脉仍显示进行性扩张。在疾病的第 28 天开始静脉注射英夫利昔单抗后，才开始出现退热的治疗反应，发热在 24 小时内得到缓解。在疾病的第 40 天，他出现了手脚小关节关节炎，只有在开始甲氨蝶呤治疗后才得到缓解。高血压在疾病发作后持续了 4 个月才完全缓解。
本病例报告描述了一例不典型表现的 KD，伴有水疱状斑片状银屑病样皮疹、高血压和迟发性小关节关节炎。它强调了临床医生应该意识到 KD 可能表现出如此不典型的表现，并可能出现不寻常的并发症。
