Abstract
Acute generalized exanthematous pustulosis (AGEP) is a rare but serious adverse skin reaction usually caused by drugs. We reported a 4-year-old boy diagnosed with Kawasaki disease (KD) accompanied by AGEP who presented with typical symptoms of KD, including fever, scarlet-like rash, swollen extremities, cervical lymph node swelling, conjunctival congestion, strawberry tongue, and cracked red lips on admission. On Day 5 of illness, a pustular eruption occurred and the patient was subsequently diagnosed with AGEP. After a double dose of intravenous immunoglobulin (IVIG) and treatment with methylprednisolone and aspirin, the patient recovered and had no cardiovascular complications. KD and AGEP can coexist, and pustular rash is not always a cutaneous manifestation of Kawasaki disease.