We present the case report of a 6-year-old patient who developed Stevens–Johnson syndrome (SJS) and acute vanishing bile duct syndrome (VBDS) after taking oral amoxicillin and naproxen. SJS, an immune complex-mediated hypersensitivity reaction involving the skin and mucosa, is usually drug-induced, and it can lead to systemic symptoms. Acute VBDS is rare, often presenting with progressive loss of the intrahepatic biliary tract. VBDS is an immune-mediated bile duct-associated disease, and immunological damage to the bile duct system is an important mechanism for VBDS. Serious drug-induced liver injury (DILI) is also associated with immunity. The drug acts as a hapten with keratin on the surface of biliary epithelial cells. The autoantibodies produced by this action can damage the bile duct epithelial cells and cause the bile duct to disappear. SJS is a serious type of polymorphic erythema that is mainly considered to be a hypersensitivity reaction to drugs, and it may involve multiple factors. The patient in this case report was treated with glucocorticoids, plasma exchange, ursodeoxycholic acid, and traditional Chinese medicine. He recovered completely within 5 months. This case report indicates that caution should be used because amoxicillin and naproxen can cause SJS and VBDS in children.
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