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

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.

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.

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.