title: Epidermal apoptotic cell death in erythema multiforme and Stevens-Johnson syndrome. Contribution of perforin-positive cell infiltration.
abstract: OBJECTIVE
  To clarify the mechanism of epidermal cell death in erythema multiforme.
  
  
  METHODS
  Retrospective study.
  
  
  METHODS
  Academic referral center.
  
  
  METHODS
  Nine patients with Stevens-Johnson syndrome (SJS), 9 patients with Hebra disease (EMH), and 5 healthy volunteers.
  
  
  METHODS
  Biopsy specimens were obtained from the border of the fresh lesions before treatment. Control specimens were obtained from normal skin.
  
  
  METHODS
  Histopathological epidermal apoptosis detected with nuclear DNA fragmentation and counts of dermal immunoreactive perforin-positive infiltrates were compared between SJS and EMH.
  
  
  RESULTS
  Eight patients (89%) with SJS showed clear apoptosis with keratinocyte DNA fragmentation. All SJS samples had intensive perforin-positive dermal infiltrates. Only 3 patients (33%) with EMH showed apoptotic change, and it was to a far less extent with far less dermal perforin-positive infiltrates. Control specimens showed no apoptotic cells in the epidermis or expression of perforin in the dermis.
  
  
  CONCLUSIONS
  Perforin mediates apoptosis in the pathogenesis of the epidermal cell changes in SJS but not in EMH. In addition to the differences in clinical severity and histopathological conditions, our findings indicate a pathogenic difference between SJS and EMH.
authors.affiliation: Department of Dermatology, Mie University Faculty of Medicine, Japan.
authors.forename: S
authors.initials: S
authors.lastname: Inachi
authors.forename: H
authors.initials: H
authors.lastname: Mizutani
authors.forename: M
authors.initials: M
authors.lastname: Shimizu
mesh_terms: D000911:Antibodies, Monoclonal; D017209:Apoptosis; D016923:Cell Death; D004396:Coloring Agents; D053938:DNA Fragmentation; D004253:DNA Nucleotidylexotransferase; D003856:Deoxyuracil Nucleotides; D004817:Epidermis / Q000473:pathology*; D004892:Erythema Multiforme / Q000209:etiology / Q000473:pathology*; D006801:Humans; D007124:Immunoenzyme Techniques; D015603:Keratinocytes / Q000473:pathology; D008562:Membrane Glycoproteins / Q000032:analysis* / Q000502:physiology; D054353:Perforin; D052899:Pore Forming Cytotoxic Proteins; D012189:Retrospective Studies; D012867:Skin / Q000473:pathology; D013262:Stevens-Johnson Syndrome / Q000209:etiology / Q000473:pathology*; D013602:T-Lymphocytes, Cytotoxic / Q000473:pathology*
chemical_list: D000911:Antibodies, Monoclonal; D004396:Coloring Agents; D003856:Deoxyuracil Nucleotides; D008562:Membrane Glycoproteins; D052899:Pore Forming Cytotoxic Proteins; C027078:deoxyuridine triphosphate; D054353:Perforin; D004253:DNA Nucleotidylexotransferase