Kawasaki Disease with Peripheral Gangrene in Children: A Case Series and Review of Literature

Kawasaki disease (KD) is a medium vessel vasculitis of childhood particularly affecting the coronary arteries and leads to aneurysms in 15-25% of untreated patients. It is a rare cause of peripheral limb gangrene in children under 5 years. Most of the cases in literature are reported during infancy. The median age of KD in patients with gangrene is 3 months and the median time for onset of signs of ischaemic necrosis is 2-4 weeks after the diagnosis and often leads to amputation of the affected limb. Even with early treatment, salvaging the limb is difficult and often not successful. There is an acute risk of mortality and long-term morbidity if the underlying inflammatory process is not identified and treated in a timely manner. We present four cases of KD, who had peripheral limb gangrene at the time of presentation. Initial workup for infective aetiology and pro-coagulant state were negative. Diagnosis of peripheral limb gangrene secondary to atypical KD was made on the basis of echocardiography and laboratory findings.