title: Plasmapheresis as adjuvant therapy in Stevens-Johnson syndrome and hepatic encephalopathy.
abstract: Stevens-Johnson syndrome (SJS) is a severe idiosyncratic reaction, most commonly triggered by medications, which is characterized by fever and mucocutaneous lesions, leading to necrosis and sloughing of the epidermis. Aside from skin and mucosal manifestations, SJS may also compromise heart, liver, kidney, lung, and gastrointestinal tract. Although cholestatic liver disease has been reported to occur in SJS, hepatic encephalopathy (HE) as a delayed complication has never been reported. We report a 4-year-old female child with anticonvulsant-induced SJS complicated by HE who was completely cured with a combination of systemic corticosteroid, intravenous immunoglobulin (IVIG), and plasmapheresis therapy. We suggested that plasmapheresis may be used as an adjuvant therapy for SJS with HE.
authors.affiliation: Division of Pediatric Neurology, Chang Gung Children's Hospital, College of Medicine, Chang Gung University, Taoyuan, Taiwan. Electronic address: h2918@cgmh.org.tw.
authors.forename: Po-Cheng
authors.initials: PC
authors.lastname: Hung
authors.affiliation: Division of Pediatric Neurology, Chang Gung Children's Hospital, College of Medicine, Chang Gung University, Taoyuan, Taiwan.
authors.forename: Huei-Shyong
authors.initials: HS
authors.lastname: Wang
authors.affiliation: Division of Pediatric Critical Care, Chang Gung Children's Hospital, College of Medicine, Chang Gung University, Taoyuan, Taiwan.
authors.forename: Shao-Hsuan
authors.initials: SH
authors.lastname: Hsia
authors.affiliation: Division of Pediatric Neuroradiology, Chang Gung Children's Hospital, College of Medicine, Chang Gung University, Taoyuan, Taiwan.
authors.forename: Alex M-C
authors.initials: AM
authors.lastname: Wong
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000927:Anticonvulsants / Q000009:adverse effects; D001921:Brain / Q000473:pathology / Q000503:physiopathology; D002675:Child, Preschool; D004569:Electroencephalography; D005260:Female; D006501:Hepatic Encephalopathy / Q000150:complications* / Q000473:pathology / Q000503:physiopathology / Q000628:therapy*; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007155:Immunologic Factors / Q000627:therapeutic use; D008279:Magnetic Resonance Imaging; D010956:Plasmapheresis; D013262:Stevens-Johnson Syndrome / Q000150:complications* / Q000473:pathology / Q000503:physiopathology / Q000628:therapy*
chemical_list: D000305:Adrenal Cortex Hormones; D000927:Anticonvulsants; D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors
keywords: Child; Hepatic encephalopathy; Plasmapheresis; Stevens–Johnson