title: Beneficial effect of plasma exchange in the treatment of toxic epidermal necrolysis: a series of four cases.
abstract: BACKGROUND
  Toxic epidermal necrolysis (TEN) is a rare, life-threatening disease with a high mortality rate that is linked to drug toxicity. There is a lack of data about the underlying pathophysiologic mechanisms and treatment options. The only widely accepted treatment of TEN is withdrawal of the offending drug followed by supportive care. The potential roles of corticosteroids, intravenous immunoglobulin (IVIG) and plasmapheresis (TPE) remain controversial.
  
  
  OBJECTIVE
  We present four patients with severe TEN (all with >80% involvement of body surface) who were treated with TPE following unsuccessful treatment with corticosteroids/IVIG.
  
  
  METHODS
  TPE was performed using a COBE Spectra blood cell separator. ACD-A was used as anticoagulant fluid and the target-washed plasma volume was one body volume. Plasma was replaced by a 5% solution of human albumin + Ringer's lactate.
  
  
  RESULTS
  The mean number of TPE sessions was 5.25 ± 2.22 (range 3-8). Drugs were implicated as an etiologic agent in each case. TPE led to prompt improvement of acute condition and general health as well as halting of disease progression. Additionally, the restoration of the epithelium began in all four patients.
  
  
  CONCLUSIONS
  Plasmapheresis should be considered as an alternative treatment modality for patients with the most severe form of TEN if initial treatment with other agents, including corticosteroids and/or IVIG, fails. Drugs were suspected to be the cause of TEN in all four cases.
authors.affiliation: 2nd Department of Internal Medicine, Hematology, Charles University, Faculty of Medicine and Teaching Hospital, Hradec Králové, Czech Republic.
authors.forename: Milan
authors.initials: M
authors.lastname: Koštál
authors.forename: Milan
authors.initials: M
authors.lastname: Bláha
authors.forename: Miriam
authors.initials: M
authors.lastname: Lánská
authors.forename: Marie
authors.initials: M
authors.lastname: Koštálová
authors.forename: Vladimír
authors.initials: V
authors.lastname: Bláha
authors.forename: Eva
authors.initials: E
authors.lastname: Štepánová
authors.forename: Jaroslav
authors.initials: J
authors.lastname: Malý
mesh_terms: D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D010951:Plasma Exchange; D010956:Plasmapheresis; D012867:Skin / Q000473:pathology; D013262:Stevens-Johnson Syndrome / Q000473:pathology / Q000628:therapy*; D016896:Treatment Outcome
chemical_list: D000305:Adrenal Cortex Hormones; D016756:Immunoglobulins, Intravenous