Acute cholestasis as uncommon onset of Kawasaki disease: a case report

Abstract

Background Kawasaki disease (KD) or mucocutaneous lymph node syndrome is a vasculitis that mostly occurs in young children. Adult-onset KD (AKD) is rare and often misdiagnosed. Here we report a rare case of KD with cholestasis as principal symptom. Case presentation A 43-year-old caucasian man was admitted to our hospital for high fever, lack of appetite related to nausea and vomiting, headache and significant malaise. Physical examination highlighted fever, increasing jaundice, bilateral laterocervical lymph nodes, erythema of the palms, and strikingly red lips and conjunctiva. The clinical course was complicated by arterial hypotension, tachycardia, decreasing haemoglobin, increasing acute phase reactants tests, and multiorgan failure. Due to cardiovascular instability the patient was admitted to the local Intensive Care Unit. Chest X-ray, abdominal ultrasound, chest and abdominal CT and Colangio Magnetic Resonance were normal. Jaundice was investigated and infections, autoimmune diseases or drugs adverse reactions, were excluded. Also coronary artery computed tomography was carried out excluding coronary artery aneurysms. Broad-spectrum antibiotics were not effective. After exclusion other possible conditions, diagnosis of KD was set. He was treated with high doses of corticosteroids and acetylsalicylic acid and clinical conditions as well as laboratory exams improved. Conclusions This report dealing with an adult onset of atypical KD may be of benefit to physicians of various specialties, including primary care doctors, hospital internists, intensivists and gastroenterologists due to its peculiarities. It demonstrates that a case of prolonged fever unresponsive to antibiotics and related to cholestatic jaundice, oedema or erythema of the extremity associated with desquamation of feet and hands, and red eyes, may suggest atypical form of KD.

A Case of Adult-Onset Kawasaki Disease in an Adolescent Occurring during the COVID-19 Pandemic

“…The patient's age and the occurrence of cholestatic hepatopathy were consistent with the diagnosis of adult-onset KD rather than classic childhood KD. Cholestatic liver involvement is a well-established feature of adult-onset KD 12 13 14 15 and was found in 23% of patients in the largest case series reported to date. 16 Splenic artery vasculitis has also been described in adult-onset KD.…”

Kawasaki disease in adolescence and adulthood: Report of four cases

“…10 Cervical adenopathy, hepatitis, and arthralgia are more commonly seen in adults than in children. 15 Most pediatricians are familiar with KD.…”