Stevens-Johnson syndrome

Stevens-Johnson syndrome

Infobox_Disease
Name = Stevens-Johnson syndrome


Caption =
DiseasesDB = 4450
ICD10 = ICD10|L|51|1|l|50
ICD9 = ICD9|695.1
ICDO =
OMIM =
MedlinePlus = 000851
eMedicineSubj = emerg
eMedicineTopic = 555
eMedicine_mult = eMedicine2|derm|405
MeshID = D013262

Stevens-Johnson syndrome (SJS) is a life-threatening condition affecting the skin in which cell death causes the epidermis to separate from the dermis. The syndrome is thought to be a hypersensitivity complex affecting the skin and the mucous membranes. Although the majority of cases are idiopathic, the main class of known causes is medications, followed by infections and (rarely) cancers.

Classification

There is agreement in the medical literature that Stevens-Johnson syndrome can be considered a milder form of toxic epidermal necrolysis (TEN). However, there is debate whether it falls on a spectrum of disease that includes erythema multiforme and erythema multiforme major (also known as erythema multiforme majus). [cite journal |author=Carrozzo M, Togliatto M, Gandolfo S |title= [Erythema multiforme. A heterogeneous pathologic phenotype] |journal=Minerva Stomatol |volume=48 |issue=5 |pages=217–26 |year=1999 |pmid=10434539] [cite journal |author=Farthing P, Bagan J, Scully C |title=Mucosal disease series. Number IV. Erythema multiforme |journal=Oral Dis |volume=11 |issue=5 |pages=261–7 |year=2005 |pmid=16120111] [cite journal |author=Bastuji-Garin S, Rzany B, Stern RS, Shear NH, Naldi L, Roujeau JC |title=Clinical classification of cases of toxic epidermal necrolysis, Stevens-Johnson syndrome, and erythema multiforme |journal=Archives of dermatology |volume=129 |issue=1 |pages=92–6 |year=1993 |pmid=8420497 | doi = 10.1001/archderm.129.1.92 ] [Ogundele O, Silverberg MA. Erythema Multiforme. eMedicine.com. URL: [http://www.emedicine.com/emerg/topic173.htm http://www.emedicine.com/emerg/topic173.htm] . Accessed on: May 6, 2007.] cite journal
author = Auquier-Dunant A, Mockenhaupt M, Naldi L, Correia O, Schröder W, Roujeau JC
title = Correlations between clinical patterns and causes of erythema multiforme majus, Stevens-Johnson syndrome, and toxic epidermal necrolysis: results of an international prospective study
journal = Arch Dermatol
volume = 138
issue = 8
pages = 1019–24
year = 2002
month = August
pmid = 12164739
doi =
url = http://archderm.ama-assn.org/cgi/pmidlookup?view=long&pmid=12164739
issn = ]

Epidemiology

SJS is a rare condition, with a reported incidence of around one case per million people per year. In the United States, there are about 300 new diagnoses per year.

Causes

SJS can be caused by infections (usually following viral infections such as herpes simplex virus, influenza, mumps, cat-scratch fever, histoplasmosis, Epstein-Barr virus, or similar), allergic reactions to drugs, (Dicloflex, fluconazole, [http://www.medsafe.govt.nz/profs/Datasheet/d/DiflucanOnecap.htm Medsafe Data Sheet] March 8, 2005. Accessed April 26, 2007.] valdecoxib, sitagliptin, penicillins, barbiturates, sulfas, phenytoin, Azithromycin, Modafinil [ [http://www.fda.gov/medwatch/safety/2007/safety07.htm#Provigil US FDA 2007 Safety Alerts for Drugs, Biologics, Medical Devices, and Dietary Supplements] ] Fact|date=May 2008, lamotrigine, nevirapine, Ibuprofen [cite journal |author=Raksha MP, Marfatia YS |title=Clinical study of cutaneous drug eruptions in 200 patients |journal=Indian J Dermatol Venereol Leprol |volume=74 |issue=1 |pages=80 |year=2008 |pmid=18193504] , ethosuximide, carbamazepine) [cite journal |author=Fagot J, Mockenhaupt M, Bouwes-Bavinck J, Naldi L, Viboud C, Roujeau J |title=Nevirapine and the risk of Stevens-Johnson syndrome or toxic epidermal necrolysis |journal=AIDS |volume=15 |issue=14 |pages=1843–8 |year=2001 |pmid=11579247 | doi = 10.1097/00002030-200109280-00014 ] [cite journal |author=Devi K, George S, Criton S, Suja V, Sridevi P |title=Carbamazepine--the commonest cause of toxic epidermal necrolysis and Stevens-Johnson syndrome: a study of 7 years |journal=Indian J Dermatol Venereol Leprol |volume=71 |issue=5 |pages=325–8 |year=2005 |pmid=16394456 |url=http://www.ijdvl.com/article.asp?issn=0378-6323;year=2005;volume=71;issue=5;spage=325;epage=328;aulast=Devi] , malignancy (carcinomas and lymphomas), or idiopathic factors (up to 50% of the time). SJS has also been consistently reported as an uncommon side effect of herbal supplements containing ginseng. SJS may also be caused by cocaine usage. Stevens-Johnson Syndrome - eMedicine|emerg|555]

Although Stevens Johnson Syndrome can be caused by viral infections, malignancies or severe allergic reactions to medication, the leading cause appears to be the inappropriate use of antibiotics and sulfa drugs. Medications that have traditionally been known to lead to Stevens Johnson Syndrome, Erythema Multiforme, Lyell's Syndrome, and Toxic Epidermal Necrolysis include sulfonamides (antibiotics), penicillins (antibiotics), barbiturates (sedatives), lamotrigine (anticonvulsant), and phenytoin - Dilantin (anticonvulsant). Combining lamotrigine with sodium valproate increases the risk of Stevens-Johnson Syndrome occurring.

Genetics

In East Asians the Carbamazepine and phenytoin induced syndrome is strongly associated with HLA-B75 (B*1502 allele of HLA-B).cite journal |author=Chung WH, Hung SI, Hong HS, "et al" |title=Medical genetics: a marker for Stevens-Johnson syndrome |journal=Nature |volume=428 |issue=6982 |pages=486 |year=2004 |month=April |pmid=15057820 |doi=10.1038/428486a |url=] cite journal |author=Locharernkul C, Loplumlert J, Limotai C, "et al" |title=Carbamazepine and phenytoin induced Stevens-Johnson syndrome is associated with HLA-B*1502 allele in Thai population |journal=Epilepsia |volume= |issue= |pages= |year=2008 |month=July |pmid=18637831 |doi=10.1111/j.1528-1167.2008.01719.x |url=] cite journal |author=Man CB, Kwan P, Baum L, "et al" |title=Association between HLA-B*1502 allele and antiepileptic drug-induced cutaneous reactions in Han Chinese |journal=Epilepsia |volume=48 |issue=5 |pages=1015–8 |year=2007 |month=May |pmid=17509004 |doi=10.1111/j.1528-1167.2007.01022.x |url=] A study in Europe suggested that the gene-marker is only relevant for East Asians.cite journal |author=Alfirevic A, Jorgensen AL, Williamson PR, Chadwick DW, Park BK, Pirmohamed M |title=HLA-B locus in Caucasian patients with carbamazepine hypersensitivity |journal=Pharmacogenomics |volume=7 |issue=6 |pages=813–8 |year=2006 |month=September |pmid=16981842 |doi=10.2217/14622416.7.6.813 |url=] cite journal |author=Lonjou C, Thomas L, Borot N, "et al" |title=A marker for Stevens-Johnson syndrome ...: ethnicity matters |journal=Pharmacogenomics J. |volume=6 |issue=4 |pages=265–8 |year=2006 |pmid=16415921 |doi=10.1038/sj.tpj.6500356 |url=] Based on the Asian findings similar studies in Europe were performed, sixty-one percent of allopurinol-induced SJS/TEN carried the HLA-B58 (B*5801 allele - Phenotype frequency in Europeans is typically 3%), indicating that while the risk alleles differ between ethnicities, the HLA-B is strongly associated or closely linked to a strongly associated gene.cite journal |author=Lonjou C, Borot N, Sekula P, "et al" |title=A European study of HLA-B in Stevens-Johnson syndrome and toxic epidermal necrolysis related to five high-risk drugs |journal=Pharmacogenet. Genomics |volume=18 |issue=2 |pages=99–107 |year=2008 |month=February |pmid=18192896 |doi=10.1097/FPC.0b013e3282f3ef9c |url=]

Treatment

All medications should be discontinued, particularly those known to cause SJS reactions. Treatment is initially similar to that of patients with thermal burns, and continued care can only be supportive (e.g. IV fluids) and symptomatic (e.g. analgesic mouth rinse for mouth ulcer); there is no specific drug treatment (2002). Treatment with corticosteroids is controversial since it might aggravate the condition or increase risk of secondary infections. Other agents have been used, including cyclophosphamide and cyclosporine, but none have exhibited much therapeutic success. Intravenous immunoglobulin (IVIG) treatment has shown some promise in reducing the length of the reaction and improving symptoms. Other common supportive measures include the use of topical pain anesthetics and antiseptics, maintaining a warm environment, and intravenous analgesics. An ophthalmologist should be consulted immediately, as SJS frequently causes the formation of scar tissue inside the eyelids leading to corneal vascularization and impaired vision, as well as a host of other ocular problems. Also, an extensive physical therapy program ensues after the patient is discharged from the hospital.

Prognosis

SJS proper (with less than 10% of body surface area involved) has the mortality rate of around 5%. The risk for death can be estimated using the SCORTEN scale, which takes a number of prognostic indicators into account. Other outcomes include organ damage and blindness.

Eponym

It is named for Dr. Albert Mason Stevens and Dr. Frank Chambliss Johnson, American pediatricians who jointly published a description of the disorder in 1922. [WhoNamedIt|synd|1501|Stevens-Johnson syndrome] [A. M. Stevens, F. C. Johnson. A new eruptive fever associated with stomatitis and ophthalmia; report of two cases in children. American Journal of Diseases of Children, Chicago, 1922, 24: 526-533.] [ [http://dictionary.reference.com/browse/Stevens-Johnson%20syndrome Stevens-Johnson syndrome - Definitions from Dictionary.com ] ]

People with Stevens-Johnson Syndrome

*Padma Lakshmi, actress, model, television personality, and cookbook writer [Jess Cartner-Morley, [http://www.guardian.co.uk/weekend/story/0,,1748147,00.html "Beautiful and Damned"] , The Guardian, 8 April 2006]
*Tessa Keller of MTV show [Source: Daly, Melissa. "My Friend Ditched Me!" Seventeen Magazine Dec. 2006: 102]
*Sebastian Tamayo -- Donald Trump's Assistant
*Sabrina Brierton Johnson, whose family sued the manufacturer of Children's Motrin, Johnson & Johnson. [ [http://www.boston.com/business/healthcare/articles/2008/07/18/jury_finds_for_jj_in_motrin_suit/ Jury finds for J&J in Motrin suit] ]

References

External links

* [http://www.sjsupport.org Stevens Johnson Syndrome Foundation]
* [http://www.avimedi.net/en Association of victims of medicines]
* [http://www.amalyste.fr SJS & Lyell Francophone Patient Association]


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