title: Immunologic Mediators in Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis.
abstract: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are a spectrum of T-cell mediated immune disorders. While the contributory mechanisms leading to the apoptosis of epidermal cells in SJS/TEN remain unproven, the keratinocyte apoptosis seen in SJS/TEN is thought to occur through the T-cell mediated Fas-Fas ligand (FasL), perforin/granzyme B, and other immune mediators. Most recently, emphasis has been placed on the granulysin pathway as being the primary mediator of apoptosis and widespread epidermal necrosis in SJS/TEN. This article aims to review the proposed mechanisms by which these pathways work and the immunomodulatory therapies that have been developed in an attempt to target them.
authors.affiliation: a Massachusetts Eye and Ear Infirmary, Department of Ophthalmology, Harvard Medical School , Boston , Massachusetts , USA.
authors.forename: Hajirah N
authors.initials: HN
authors.lastname: Saeed
authors.affiliation: a Massachusetts Eye and Ear Infirmary, Department of Ophthalmology, Harvard Medical School , Boston , Massachusetts , USA.
authors.forename: James
authors.initials: J
authors.lastname: Chodosh
mesh_terms: D017209:Apoptosis; D003229:Conjunctival Diseases / Q000276:immunology / Q000473:pathology; D003316:Corneal Diseases / Q000276:immunology / Q000473:pathology; D005141:Eyelid Diseases / Q000276:immunology / Q000473:pathology; D053222:Fas Ligand Protein / Q000276:immunology*; D020397:Goblet Cells / Q000276:immunology / Q000473:pathology; D053804:Granzymes / Q000276:immunology*; D006801:Humans; D008537:Meibomian Glands / Q000276:immunology / Q000473:pathology; D054353:Perforin / Q000276:immunology*; D013262:Stevens-Johnson Syndrome / Q000276:immunology* / Q000473:pathology; D013602:T-Lymphocytes, Cytotoxic / Q000276:immunology*
chemical_list: C505661:FASLG protein, human; D053222:Fas Ligand Protein; D054353:Perforin; D053804:Granzymes
keywords: Fas; granulysin; granzyme B; immunomodulators; ocular immunology; perforin