Acute cholestasis as uncommon onset of Kawasaki disease: a case report
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CASE REPORT
Acute cholestasis as uncommon onset of Kawasaki disease: a case report Massimo Gallerani1*, Marco Pala2, Fabio Fabbian3 and Alfredo De Giorgi2
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. Keywords: Kawasaki disease, Mucocutaneous lymph node syndrome, Acute cholestasis, Jaundice Background Kawasaki disease (KD) also defined mucocutaneous lymph node syndrome is an acute multi-systemic necrotizing vasculitis that affects middle and small sized vessels and occurs predominantly in young children. KD is more frequent (80% of cases) in young patients with an age between 6 months and 4 years. Being infrequent in Caucasian children, KD is very often misdiagnosed in *Correspondence: [email protected] 1 Department of Medicine, Unit of Internal Medicine, Azienda Ospedaliera-Universitaria (AOU) of Ferrara, Via Aldo Moro 8, 44124 Cona, Ferrara, Italy Full list of author information is available at the end of the article
European adult males [1–3]. As well as in other systemic diseases non-specific symptoms are common in the 10 days before diagnosis. The suspect of KD is related to persistent fever (for more than 5 days), unresponsive to broad-spectrum antibiotics and with absence of p
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