Kawasaki Disease with Coronary Involvement
Year: 2025
Type: article
Study design: Case Report
Abstract: Kawasaki disease (KD) is a medium vessel vasculitis that most commonly affects children between 6 months to 5 years.[1] The term KD was coined by Japanese pediatrician Tomisaku Kawasaki in 1967.[2] Main symptoms include fever, conjunctivitis, skin and mucous membrane involvement, and cervical lymphadenopathy.[3] Here, we report a case of KD with five of six features fitting into the diagnostic criteria of KD. A 4-year-old febrile male child presented with redness, cracked lips, and painful swelling over the right side of the neck for 5 days which did not respond to antibiotics. Mucocutaneous examination showed bilateral (B/L) nonpurulent conjunctivitis with perilimbal sparing [Figure 1a], red inflamed (strawberry) tongue with prominent papillae, erythema with fissures over both lips [Figure 1b and c], and right tender cervical lymphadenopathy [Figure 1d]. Further investigations revealed elevated erythrocyte sedimentation rate (ESR), positive C-reactive protein (CRP), hypoalbuminemia, leukocytosis, and normocytic normochromic anemia. Electrocardiography (ECG) showed sinus tachycardia. Echocardiography (ECHO) showed borderline dilated right coronary artery (RCA) [Figure 1e] and left main coronary artery (LMCA) [Figure 1f]. The patient was started on aspirin and intravenous immunoglobulin followed by which fever reduced, and the patient’s general condition improved. KD is a medium vessel vasculitis that commonly affects children between 6 months to 5 years. Delay in diagnosis and treatment can result in coronary artery aneurysm, so early diagnosis and prompt management of KD prevents the morbidity and mortality.Figure 1: (a and c) Erythema with fissures over both lips and bilateral conjunctival congestion with perilimbal sparing. (b) shows red inflammed tongue with prominent papillae. (d) Single tender right cervical lymphadenopathy. (e and f) Dilated right coronary artery (blue arrow) and dilated left main coronary artery (green arrow)KD occurs primarily in young children, most commonly <5 years of age.[3] For any young children presenting with persistent fever not responding to conventional treatment, the possibility of KD should be considered. KD is classified into acute phase, subacute phase, and convalescent phase.[4] In the acute phase, the child may develop abrupt onset of fever, irritability, cervical lymphadenitis, conjunctivitis, changes in buccal and oral mucosa, erythema and edema in hands and feet, and pleomorphic rash.[4] In the subacute phase, desquamation of the skin of digits may be the clinically apparent residual finding. In the convalescent phase, most children may become asymptomatic, and beau’s lines may appear. Criteria for KD include fever of at least 5 days and at least 4 of the following five clinical features: (1) Polymorphic exanthem, (2) B/L nonexudative conjunctival ingestion, (3) Changes in lips and oral cavity, (4) Changes in extremities (erythema or edema or desquamation), and (5) Cervical lymphadenopathy, often unilateral and large (>1.5 cm).[5] Patients fulfilling all these criteria will be termed as complete KD. Patients who failed to meet this diagnostic criterion will be termed as incomplete KD. KD is clinical diagnosis with laboratory features such as leukocytosis, anemia for age, platelet >450,000, raised ESR, CRP, hypoalbuminemia, elevated alanine transaminase and gamma-glutamyltransferase, and >10 white blood cells in urine with ECG and ECHO showing features of coronary artery lesions.[2] Early treatment is required to prevent the coronary complications. The differential diagnosis for Kawasaki includes measles, scarlet fever, cervical lymphadenitis, staphylococcal scalded skin syndrome, toxic shock syndrome, Stevens–Johnson syndrome, drug reactions, rheumatic fever, and juvenile idiopathic arthritis which was ruled out with classical clinical features of KD in our case.[2] Treatment includes primarily intravenous immunoglobulin, most effective when administered within 10 days of onset of fever which reduces the risk of coronary artery aneurysm formation from 20% to 25% to 3%–5% with dose of 2 g/kg. Then, aspirin 30–50 mg/kg/day or high dose of 80–100 mg/kg/day can be started. Other options include corticosteroids, plasma exchange, tumor necrosis factor alpha inhibitors, cyclophosphamide, and statins.[2] In our case, the patient had fever for >5 days, lip changes, cervical lymphadenopathy, and B/L nonpurulent conjunctivitis, and there was also elevated CRP and ESR, leukocytosis, hypoalbuminemia, and borderline dilated LMCA and RCA on ECHO. With all these features, the diagnosis of KD was made since fever for >5 days with 4 of 5 criteria fitting into the diagnosis. KD is an important disease which should not be missed in children presenting with fever for >5 days because delay in treatment can lead to coronary artery aneurysm, and its sequelae include cardiac infarctions. Hence, early diagnosis and prompt management of KD prevent the morbidity and mortality. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Authors’ contributions Literature search: K.V, M.K; Data acquisition: B.S, Y.S; Manuscript preparation: B.S, Y.S; Manuscript editing: K.V, M.K, B.S, Y.S; Manuscript review: B.S, Y.S. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.
Source: Clinical Dermatology Review
Authors: Kovalan Vishwanath, Manobalan Karunanandhan, Baskaran Srinivasan, Yogindher Singh
Language: English
Subfield: Surgery
Field: Medicine
Domain: Health Sciences
Open Access status: diamond
OpenAlex ID: W4409185534