title: Sulthiame-induced drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome.
abstract: BACKGROUND
  Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a rare and potentially life-threatening acute drug-induced hypersensitivity reaction. Antiepileptic drugs (AEDs) predominantly aromatic AEDs are commonly reported in DRESS. To date there are no reports of sulthiame AED causing DRESS syndrome.
  
  
  METHODS
  We report a 10-year-old girl of Indian descent with AED resistant epilepsy on maintenance sodium valproate and clonazepam. Sulthiame AED was initiated to try to improve her seizure control. Five weeks after commencing sulthiame, she developed fever with a diffuse erythematous morbilliform maculopapular rash, elevated transaminases and atypical lymphocytes. At day 3 of illness, she deteriorated with worsening elevation of liver transaminases, thrombocytopenia, progression of rash, hepatosplenomegaly, pneumonitis and markedly elevated inflammatory markers. Immunomodulatory treatment of pulse methylprednisolone was given from day 7 which was associated with improvement inflammatory markers and complete resolution of rash from day 30 of illness.
  
  
  RESULTS
  The diagnosis of sulthiame-induced DRESS syndrome was made based on clinical, laboratory and skin histology findings. She was HLA-B heterozygous for HLA-B∗15:123 and 15:240 and HLA-A homozygous for HLA-A∗11:01:09. Both these HLA-A and HLA-B typing has not been reported before in cutaneous drug reactions.
  
  
  CONCLUSIONS
  This is the first reported case of sulthiame-induced DRESS syndrome. Our case expands the list of possible susceptible HLA alleles associated with cutaneous drug reactions. It also raises the awareness of possible DRESS syndrome among patients commenced on sulthiame who will require immediate discontinuation of sulthiame and consideration of prompt treatment of corticosteroids.
authors.affiliation: Division of Paediatric Neurology, Department of Paediatrics, University of Malaya, Kuala Lumpur, Malaysia. Electronic address: cyfong@ummc.edu.my.
authors.forename: Choong Yi
authors.initials: CY
authors.lastname: Fong
authors.affiliation: Division of Paediatric Neurology, Department of Paediatrics, University of Malaya, Kuala Lumpur, Malaysia.
authors.forename: Nurmaira
authors.initials: N
authors.lastname: Hashim
authors.affiliation: Division of Paediatric Intensive Care, Department of Paediatrics, University of Malaya, Kuala Lumpur, Malaysia.
authors.forename: Chin Seng
authors.initials: CS
authors.lastname: Gan
authors.affiliation: Department of Pathology, University of Malaya, Kuala Lumpur, Malaysia.
authors.forename: Tak Kuan
authors.initials: TK
authors.lastname: Chow
authors.affiliation: Division of Paediatric Neurology, Department of Paediatrics, University of Malaya, Kuala Lumpur, Malaysia.
authors.forename: Chee Geap
authors.initials: CG
authors.lastname: Tay
mesh_terms: D000483:Alleles; D000893:Anti-Inflammatory Agents / Q000008:administration & dosage / Q000627:therapeutic use; D000927:Anticonvulsants / Q000009:adverse effects*; D002648:Child; D063926:Drug Hypersensitivity Syndrome / Q000503:physiopathology*; D000069279:Drug Resistant Epilepsy / Q000150:complications; D005260:Female; D005334:Fever / Q000139:chemically induced; D006650:Histocompatibility Testing; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D008775:Methylprednisolone / Q000008:administration & dosage / Q000627:therapeutic use; D013843:Thiazines / Q000009:adverse effects*; D000096926:Benzenesulfonamides
chemical_list: D000893:Anti-Inflammatory Agents; D000927:Anticonvulsants; D016756:Immunoglobulins, Intravenous; D008775:Methylprednisolone; D013843:Thiazines; C084593:sulthiame; D000096926:Benzenesulfonamides
keywords: Adverse drug reaction; Antiepileptic drug; DRESS syndrome; Hepatitis; Sulthiame