Complete Kawasaki Disease in a Child with Transient Hypogammaglobulinemia of Infancy

Abstract

Kawasaki disease is an acute febrile illness of early childhood characterized by vasculitis of the arteries. The diagnosis of complete Kawasaki disease should be made in a child who has a fever lasting 5 days or more and has at least 4 of 5 clinical criteria: rash, conjunctival injection, oropharyngeal erythema, swelling and erythema of the extremities, and unilateral cervical lymphadenopathy. Incomplete form of the disease is diagnosed when a patient presents with fever for 5 days or longer, 2 or 3 of the principal clinical features, and laboratory findings suggestive of the disease or echocardiographic abnormalities. Kawasaki disease has been described as a complication of various primary and secondary immunodeficiency disorders thus supporting an infectious etiology of this disease. Immunodeficiencies may result in an incomplete clinical presentation of Kawasaki disease and end up with delay in diagnosis and therefore treatment, which may lead to development of coronary artery aneurism . We are presenting a 2,5 year old girl with transient hypogammaglobulinemia of infancy who has complete form of the disease without coronary artery aneurism development , to emphasize the occurrence of Kawasaki disease in immune deficiency situations.