Scoring Assessments in Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis
Epidermal necrolysis, the unifying term for Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN), is a severe cutaneous drug reaction associated with high morbidity and mortality. Given the rarity of this disease, large-scale prospective research studies are limited. Significant insti...
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Frontiers Media S.A.
2022-06-01
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| Series: | Frontiers in Medicine |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fmed.2022.883121/full |
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| author | Allison S. Dobry Sonia Himed Margo Waters Benjamin H. Kaffenberger Benjamin H. Kaffenberger |
| author_facet | Allison S. Dobry Sonia Himed Margo Waters Benjamin H. Kaffenberger Benjamin H. Kaffenberger |
| author_sort | Allison S. Dobry |
| collection | DOAJ |
| description | Epidermal necrolysis, the unifying term for Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN), is a severe cutaneous drug reaction associated with high morbidity and mortality. Given the rarity of this disease, large-scale prospective research studies are limited. Significant institutional and geographical variations in treatment practices highlight the need for standardization of clinical assessment scores and prioritization of research outcome measures in epidermal necrolysis. At the present, clinical assessment is typically simplified to total body surface area (BSA) involvement, with little focus on morphology. Validated clinical scoring systems are used as mortality prognostication tools, with SCORTEN being the best-validated tool thus far, although the ABCD-10 has also been recently introduced. These tools are imperfect in that they tend to either overestimate or underestimate mortality in certain populations and are not designed to monitor disease progression. Although mortality is often used as a primary endpoint for epidermal necrolysis studies, this outcome fails to capture more nuanced changes in skin disease such as arrest of disease progression while also lacking a validated skin-directed inclusion criterion to stratify patients based on the severity of skin disease at study entry. In addition to mortality, many studies also use BSA stabilization or time to re-epithelialization as endpoints, although these are not clearly defined morphologically, and inter- and intra-rater reliability are unclear. More specific, validated cutaneous assessment scores are necessary in order advance therapeutic options for epidermal necrolysis. In this review, we summarize the strengths and weaknesses of current clinical assessment practices in epidermal necrolysis and highlight the need for standardized research tools to monitor cutaneous involvement throughout the hospitalization. |
| first_indexed | 2024-04-13T17:53:56Z |
| format | Article |
| id | doaj.art-0c3574d86132499eb7652a758384e481 |
| institution | Directory Open Access Journal |
| issn | 2296-858X |
| language | English |
| last_indexed | 2024-04-13T17:53:56Z |
| publishDate | 2022-06-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Medicine |
| spelling | doaj.art-0c3574d86132499eb7652a758384e4812022-12-22T02:36:35ZengFrontiers Media S.A.Frontiers in Medicine2296-858X2022-06-01910.3389/fmed.2022.883121883121Scoring Assessments in Stevens-Johnson Syndrome and Toxic Epidermal NecrolysisAllison S. Dobry0Sonia Himed1Margo Waters2Benjamin H. Kaffenberger3Benjamin H. Kaffenberger4Department of Dermatology, University of California, San Francisco, San Francisco, CA, United StatesDivision of Dermatology, Ohio State University Wexner Medical Center, Columbus, OH, United StatesOhio State University College of Medicine, Columbus, OH, United StatesDivision of Dermatology, Ohio State University Wexner Medical Center, Columbus, OH, United StatesOhio State University College of Medicine, Columbus, OH, United StatesEpidermal necrolysis, the unifying term for Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN), is a severe cutaneous drug reaction associated with high morbidity and mortality. Given the rarity of this disease, large-scale prospective research studies are limited. Significant institutional and geographical variations in treatment practices highlight the need for standardization of clinical assessment scores and prioritization of research outcome measures in epidermal necrolysis. At the present, clinical assessment is typically simplified to total body surface area (BSA) involvement, with little focus on morphology. Validated clinical scoring systems are used as mortality prognostication tools, with SCORTEN being the best-validated tool thus far, although the ABCD-10 has also been recently introduced. These tools are imperfect in that they tend to either overestimate or underestimate mortality in certain populations and are not designed to monitor disease progression. Although mortality is often used as a primary endpoint for epidermal necrolysis studies, this outcome fails to capture more nuanced changes in skin disease such as arrest of disease progression while also lacking a validated skin-directed inclusion criterion to stratify patients based on the severity of skin disease at study entry. In addition to mortality, many studies also use BSA stabilization or time to re-epithelialization as endpoints, although these are not clearly defined morphologically, and inter- and intra-rater reliability are unclear. More specific, validated cutaneous assessment scores are necessary in order advance therapeutic options for epidermal necrolysis. In this review, we summarize the strengths and weaknesses of current clinical assessment practices in epidermal necrolysis and highlight the need for standardized research tools to monitor cutaneous involvement throughout the hospitalization.https://www.frontiersin.org/articles/10.3389/fmed.2022.883121/fullSJS/TENscoring assessmentdrug reactionepidermal necrolysisdermatology |
| spellingShingle | Allison S. Dobry Sonia Himed Margo Waters Benjamin H. Kaffenberger Benjamin H. Kaffenberger Scoring Assessments in Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Frontiers in Medicine SJS/TEN scoring assessment drug reaction epidermal necrolysis dermatology |
| title | Scoring Assessments in Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis |
| title_full | Scoring Assessments in Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis |
| title_fullStr | Scoring Assessments in Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis |
| title_full_unstemmed | Scoring Assessments in Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis |
| title_short | Scoring Assessments in Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis |
| title_sort | scoring assessments in stevens johnson syndrome and toxic epidermal necrolysis |
| topic | SJS/TEN scoring assessment drug reaction epidermal necrolysis dermatology |
| url | https://www.frontiersin.org/articles/10.3389/fmed.2022.883121/full |
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