Epidermal apoptotic cell death in erythema multiforme and Stevens-Johnson syndrome. Contribution of perforin-positive cell infiltration.
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.
Inachi
S
S
Department of Dermatology, Mie University Faculty of Medicine, Japan.
Mizutani
H
H
Shimizu
M
M
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*
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