156 - PERIPHERAL GANGRENE IN KAWASAKI DISEASE: A CLINICAL CONUNDRUM

Background: Kawasaki disease (KD) is a medium vessel vasculitis affecting young children. It usually affects the coronary arteries although other medium sized arteries(e.g. brachial and femoral) can also be involved and may present as peripheral aneurysms. Occasionally, peripheral arterial obstruction may lead to ischaemia and gangrene. Peripheral gangrene is an unusual complication in KD.

Patients and methods: This study was conducted in Allergy Immunology Unit,  Advanced Paediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh, India. Records of all children diagnosed with KD during 1994-2023 were analyzed.  Diagnosis of KD was based on standard guidelines . Clinical details of children who had peripheral gangrene were reviewed.

Case details: Of the 1350 children with KD diagnosed during 1994-2023, 9 ( 4 boys:5 girls) developed peripheral gangrene. All patients had had history of prolonged fever and had peri-ungual desquamation in the convalescent phase. Inflammatory markers were elevated in all the patients during the acute phase of illness. Pro-BNP was assayed in 10 patients and was found to be elevated. Four patients developed Beau’s lines. Cardiac evaluation by echocardiography showed low ejection fraction suggestive of myocarditis in 3 patients. No coronary artery abnormalities were noted in any of the patients. Other significant clinical findings included macrophage activation syndrome (1 patient),  CNS infarcts (1 patient) and acute arthritis (1patient).  Four patients received additional therapies -  methylprednisolone (in patient 1 and 4)  and infliximab (in patient 4,5 and 9).. Prothrombotic workup (Factor V Leiden, Protein C, S and anti-phospholipid antibodies) was unremarkable in all patients. All 9 patents received low molecular weight heparin (1 mg/kg twice a day; duration range 2-3 months) and oral aspirin. On follow-up gangrenous lesions improved in all  9 patients.

Discussion and conclusions: Peripheral gangrene is an uncommon finding in KD. It may occasionally be the only presenting clinical feature of KD along with prolonged fever. Paediatricians need to be aware of this unusual presentation of KD.