title: Immunological response in Stevens-Johnson syndrome and toxic epidermal necrolysis.
abstract: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening cutaneous adverse drug reactions that induce widespread epidermal necrosis. Recent advances in pharmacogenomic studies have provided evidence of genetic predispositions to SJS/TEN. Several concepts have been proposed to explain the pathogenesis of severe cutaneous adverse drug reactions. In the hapten concept, small molecules called haptens elicit an immune response only when attached to proteins. The "p-i" concept postulates that the causative drugs can stimulate cells by binding directly and reversibly to immune receptors. In addition, there is the idea that drugs alter the antigen by binding to the human leukocyte antigen pocket. With regard to keratinocyte death, several cell death mediators, such as FasL, granulysin and annexin A1, have been proposed as playing a role in SJS/TEN pathogenesis. A subset of T lymphocytes, including regulatory T cells, also may play a role in SJS/TEN.
authors.affiliation: Department of Dermatology, Hokkaido University Graduate School of Medicine, Sapporo, Japan.
authors.forename: Riichiro
authors.initials: R
authors.lastname: Abe
mesh_terms: D017305:Annexin A1 / Q000378:metabolism; D017951:Antigen Presentation; D000945:Antigens, Differentiation, T-Lymphocyte / Q000378:metabolism; D053804:Granzymes / Q000378:metabolism; D006241:Haptens / Q000276:immunology; D006801:Humans; D054353:Perforin / Q000378:metabolism; D011948:Receptors, Antigen, T-Cell / Q000378:metabolism; D044042:Receptors, Formyl Peptide / Q000378:metabolism; D013262:Stevens-Johnson Syndrome / Q000276:immunology* / Q000378:metabolism; D019014:fas Receptor / Q000378:metabolism
chemical_list: D017305:Annexin A1; D000945:Antigens, Differentiation, T-Lymphocyte; C469986:FPR1 protein, human; C075005:GNLY protein, human; D006241:Haptens; D011948:Receptors, Antigen, T-Cell; D044042:Receptors, Formyl Peptide; D019014:fas Receptor; D054353:Perforin; D053804:Granzymes
keywords: Stevens-Johnson syndrome; drug; hapten; p-i concept; toxic epidermal necrolysis