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Stevens–Johnson syndrome

From Wikipedia
Stevens–Johnson syndrome
designated intractable/rare disease, class of disease
Subclass ofskin disease, erythema, severe cutaneous adverse reactions, syndrome, disease Edit
Dem name afterAlbert Mason Stevens, Frank Chambliss Johnson, Ernst Wilhelm Baader Edit
Health specialtydermatology Edit
Genetic associationPSORS1C1, POU5F1 Edit
External data available at URLhttp://www.nanbyou.or.jp/entry/4073 Edit
ICD-9-CM695.13 Edit

Stevens–Johnson syndrome (SJS) be a type of severe skin reaction.[1] Togeda plus toxic epidermal necrolysis (TEN) den Stevens–Johnson/toxic epidermal necrolysis (SJS/TEN) overlap, dem be considered febrile mucocutaneous drug reactions den probably part of de same spectrum of disease, wey SJS be less severe.[1][2][3] Erythema multiforme (EM) generally be considered a separate condition.[4] Early symptoms of SJS dey include fever den flu-like symptoms.[1] A few days later, de skin dey begin to blister den peel, dey form painful raw areas.[1] Mucous membranes, such as de mouth, sanso typically be involved.[1] Complications dey include dehydration, sepsis, pneumonia den multiple organ failure.[1]

De most common cause be certain medications such as lamotrigine, carbamazepine, allopurinol, sulfonamide antibiotics den nevirapine.[1] Oda causes fi include infections such as Mycoplasma pneumoniae den cytomegalovirus, anaa de cause fi remain unknown.[1][5] Risk factors dey include HIV/AIDS den systemic lupus erythematosus.[1]

De diagnosis of Stevens–Johnson syndrome dey base on involvement of less dan 10% of de skin.[5] E be known as TEN wen more dan 30% of de skin be involved wey e be considered an intermediate form wen 10–30% be involved.[6] SJS/TEN reactions be believed to follow a type IV hypersensitivity mechanism.[7] E sanso be included plus drug reaction plus eosinophilia den systemic symptoms (DRESS syndrome), acute generalized exanthematous pustulosis (AGEP) den toxic epidermal necrolysis insyd a group of conditions dem know as severe cutaneous adverse reactions (SCARs).[8]

Treatment typically dey take place insyd hospital such as in a burn unit anaa intensive care unit.[5] Efforts fi include stopping de cause, pain medication, antihistamines, antibiotics, intravenous immunoglobulins anaa corticosteroids.[5] Togeda plus TEN, SJS dey affect 1 to 2 people per million per year.[1] Typical onset be under de age of 30.[5] Skin usually dey regrow over two to three weeks; however, complete recovery fi take months.[5] Overall, de risk of death plus SJS be 5 to 10%.[1][9]

Notable cases

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  • Ab-Soul, American hip-hop recording artist den member of Black Hippy[10]
  • Padma Lakshmi, actress, model, television personality, den cookbook writer[11]
  • Manute Bol, former NBA player. Bol die from complications of Stevens–Johnson syndrome as well as kidney failure.[12]
  • Gene Sauers, three-time PGA Tour winner[13]
  • Samantha Reckis, a seven-year-old Plymouth, Massachusetts girl wey loose de skin covering 95% of ein body after she take kiddies dema Motrin insyd 2003. Insyd 2013, a jury award am $63M insyd a lawsuit against Johnson & Johnson, one of de largest lawsuits of ein kind.[14] Dem uphold de decision insyd 2015.[15]
  • Karen Elaine Morton, a model den actress wey appear insyd Tommy Tutone ein "867-5309/Jenny" video.[16]

References

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  1. 1 2 3 4 5 6 7 8 9 10 11 "Stevens-Johnson syndrome/toxic epidermal necrolysis". Genetics Home Reference (in English). July 2015. Archived from the original on April 27, 2017. Retrieved April 26, 2017.
  2. ↑ Creamer, D.; Walsh, S.A.; Dziewulski, P.; Exton, L.S.; Lee, H.Y.; Dart, J.K.G.; Setterfield, J.; Bunker, C.B.; Ardern-Jones, M.R.; Watson, K.M.T.; Wong, G.A.E.; Philippidou, M.; Vercueil, A.; Martin, R.V.; Williams, G. (June 2016). "U.K. guidelines for the management of Stevens–Johnson syndrome/toxic epidermal necrolysis in adults 2016". British Journal of Dermatology (in English). 174 (6): 1194–1227. doi:10.1111/bjd.14530. ISSN 0007-0963. PMID 27317286.
  3. ↑ "Orphanet: Toxic epidermal necrolysis". Orphanet (in English). November 2008. Archived from the original on April 27, 2017. Retrieved April 26, 2017.
  4. ↑ Schwartz, RA; McDonough, PH; Lee, BW (August 2013). "Toxic epidermal necrolysis: Part I. Introduction, history, classification, clinical features, systemic manifestations, etiology, and immunopathogenesis". Journal of the American Academy of Dermatology. 69 (2): 173.e1–13, quiz 185–6. doi:10.1016/j.jaad.2013.05.003. PMID 23866878.
  5. 1 2 3 4 5 6 "Stevens-Johnson syndrome". GARD. Archived from the original on August 28, 2016. Retrieved August 26, 2016.
  6. ↑ "Orphanet: Toxic epidermal necrolysis". Orphanet (in English). November 2008. Archived from the original on April 27, 2017. Retrieved April 26, 2017.
  7. ↑ Hyzy, Robert C. (2017). Evidence-Based Critical Care: A Case Study Approach (in English). Springer. p. 761. ISBN 978-3-319-43341-7.
  8. ↑ Adler NR, Aung AK, Ergen EN, Trubiano J, Goh MS, Phillips EJ (2017). "Recent advances in the understanding of severe cutaneous adverse reactions". The British Journal of Dermatology. 177 (5): 1234–1247. doi:10.1111/bjd.15423. PMC 5582023. PMID 28256714.
  9. ↑ Lerch M, Mainetti C, Terziroli Beretta-Piccoli B, Harr T (2017). "Current Perspectives on Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis". Clinical Reviews in Allergy & Immunology. 54 (1): 147–176. doi:10.1007/s12016-017-8654-z. PMID 29188475. S2CID 46796285.
  10. ↑ Ramirez, Erika (August 8, 2012). "Ab-Soul's timeline: The rapper's life from 5 years old to now". billboard.com. Billboard. Archived from the original on February 17, 2013. Retrieved December 7, 2012.
  11. ↑ Cartner-Morley, Jess (April 8, 2006). "Beautiful and Damned". The Guardian.
  12. ↑ "Manute Bol dies at age 47". FanHouse. AOL. June 19, 2010. Archived from the original on June 21, 2010.
  13. ↑ Graff, Chad (July 31, 2013). "3M golf: Gene Sauers thriving after torturous battle with skin disease". St. Paul Pioneer Press. Archived from the original on October 3, 2014.
  14. ↑ "Family awarded $63 million in Motrin case". The Boston Globe. February 13, 2013. Archived from the original on September 5, 2017.
  15. ↑ "$63 million verdict in Children's Motrin case upheld". The Boston Globe. April 17, 2015. Archived from the original on October 31, 2016.
  16. ↑ Morton, Karen. "Karen Morton Biography". imdb.com. Archived from the original on October 2, 2016.
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