Stevens-Johnson syndrome/toxic epidermal necrolysis spectrum for the rheumatologist

Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) represent a spectrum of mucocutaneous manifestations characterized by widespread epidermal and/or mucosal detachment from dermis. It occurs due to Type IV hypersensitivity leading to keratinocyte apoptosis. Many extrinsic and intrinsic de...

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Main Authors: Jyoti Ranjan Parida, Saumya Ranjan Tripathy
Format: Article
Language:English
Published: SAGE Publishing 2019-01-01
Series:Indian Journal of Rheumatology
Subjects:
Online Access:http://www.indianjrheumatol.com/article.asp?issn=0973-3698;year=2019;volume=14;issue=5;spage=67;epage=75;aulast=Parida
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author Jyoti Ranjan Parida
Saumya Ranjan Tripathy
author_facet Jyoti Ranjan Parida
Saumya Ranjan Tripathy
author_sort Jyoti Ranjan Parida
collection DOAJ
description Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) represent a spectrum of mucocutaneous manifestations characterized by widespread epidermal and/or mucosal detachment from dermis. It occurs due to Type IV hypersensitivity leading to keratinocyte apoptosis. Many extrinsic and intrinsic defects in the apoptotic pathway have been postulated to result in its dysregulation. The characteristic skin lesions of SJS/TEN include ill-defined, coalescing, red macules with necrotic centers followed by extensive epidermal detachment. Mucosa is involved in up to 90% of cases causing erosions and crusts that may precede or follow skin lesions. Supportive care is the mainstay of treatment and if not started promptly may result in high mortality and morbidity. Although many immunosuppressive drugs have been tried in treatment, no treatment has proven beneficial except cyclosporine which has been shown to retard the progression of SJS/TEN. A patient with SJS/TEN as a presenting feature poses two-fold challenge to a rheumatologist as it could be a manifestation of lupus or side effects of a myriad of drugs used by the rheumatologist.
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spelling doaj-art-06099bfc79334db186b515f19da429262025-02-02T23:09:23ZengSAGE PublishingIndian Journal of Rheumatology0973-36980973-37012019-01-01145677510.4103/0973-3698.272155Stevens-Johnson syndrome/toxic epidermal necrolysis spectrum for the rheumatologistJyoti Ranjan ParidaSaumya Ranjan TripathyStevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) represent a spectrum of mucocutaneous manifestations characterized by widespread epidermal and/or mucosal detachment from dermis. It occurs due to Type IV hypersensitivity leading to keratinocyte apoptosis. Many extrinsic and intrinsic defects in the apoptotic pathway have been postulated to result in its dysregulation. The characteristic skin lesions of SJS/TEN include ill-defined, coalescing, red macules with necrotic centers followed by extensive epidermal detachment. Mucosa is involved in up to 90% of cases causing erosions and crusts that may precede or follow skin lesions. Supportive care is the mainstay of treatment and if not started promptly may result in high mortality and morbidity. Although many immunosuppressive drugs have been tried in treatment, no treatment has proven beneficial except cyclosporine which has been shown to retard the progression of SJS/TEN. A patient with SJS/TEN as a presenting feature poses two-fold challenge to a rheumatologist as it could be a manifestation of lupus or side effects of a myriad of drugs used by the rheumatologist.http://www.indianjrheumatol.com/article.asp?issn=0973-3698;year=2019;volume=14;issue=5;spage=67;epage=75;aulast=Paridaadverse effectsdrug inducedstevens-johnson syndrometoxic epidermal necrolysis
spellingShingle Jyoti Ranjan Parida
Saumya Ranjan Tripathy
Stevens-Johnson syndrome/toxic epidermal necrolysis spectrum for the rheumatologist
Indian Journal of Rheumatology
adverse effects
drug induced
stevens-johnson syndrome
toxic epidermal necrolysis
title Stevens-Johnson syndrome/toxic epidermal necrolysis spectrum for the rheumatologist
title_full Stevens-Johnson syndrome/toxic epidermal necrolysis spectrum for the rheumatologist
title_fullStr Stevens-Johnson syndrome/toxic epidermal necrolysis spectrum for the rheumatologist
title_full_unstemmed Stevens-Johnson syndrome/toxic epidermal necrolysis spectrum for the rheumatologist
title_short Stevens-Johnson syndrome/toxic epidermal necrolysis spectrum for the rheumatologist
title_sort stevens johnson syndrome toxic epidermal necrolysis spectrum for the rheumatologist
topic adverse effects
drug induced
stevens-johnson syndrome
toxic epidermal necrolysis
url http://www.indianjrheumatol.com/article.asp?issn=0973-3698;year=2019;volume=14;issue=5;spage=67;epage=75;aulast=Parida
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