Kawasaki disease-like syndrome (KDLS) has been described in HIV-infected individuals. However, coronary artery abnormalities have not been reported in these patients.

A 14-year-old boy with advanced perinatal HIV infection and ITP required hospital admission for fever and bilateral hand swelling of 3 days duration. Generalized erythematous rash, palmar erythema, and conjunctivitis were noted on examination. Blood, urine, and throat cultures were negative. HIV-1 RNA was 990,000 copies/mL and CD4 count was 10 cells/mm³ (1%). All medications including didanosine, stavudine, tenofovir, enfuvirtide, azithromycin, and atovaquone were discontinued for a suspected drug reaction. High fever (Tmax 104.7°F) persisted despite 5 days of intravenous nafcillin and cefotaxime. Baseline echocardiogram revealed normal coronary anatomy. A diagnosis of presumed KDLS was made. Intravenous gamma globulin (2g/kg) was administered with resolution of patient's fever and conjunctivitis within 24 hours. Aspirin therapy was not initiated secondary to ITP.

Three weeks later, ectasia of multiple coronary arteries (measuring up to 5.0 mm) was seen on echocardiogram. Desquamation was noted. One month later, a proximal saccular aneurysm of the left anterior descending artery was seen. Four months after presentation, the coronary aneurysms resolved. The patient remains well without recurrence of symptoms.

We report a case of atypical KD with coronary artery aneurysm formation in an HIV-infected adolescent. The development of KD in an extremely immunocompromised patient with ITP and lymphocytopenia implicates infection, rather than lymphocyte-dependent processes, in the pathogenesis of KD.