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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SAGE Publishing
2019-01-01
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Series: | Indian Journal of Rheumatology |
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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. |
format | Article |
id | doaj-art-06099bfc79334db186b515f19da42926 |
institution | Kabale University |
issn | 0973-3698 0973-3701 |
language | English |
publishDate | 2019-01-01 |
publisher | SAGE Publishing |
record_format | Article |
series | Indian Journal of Rheumatology |
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 |
work_keys_str_mv | AT jyotiranjanparida stevensjohnsonsyndrometoxicepidermalnecrolysisspectrumfortherheumatologist AT saumyaranjantripathy stevensjohnsonsyndrometoxicepidermalnecrolysisspectrumfortherheumatologist |