View source for Stevens-Johnson syndrome

{{Infobox_Disease |
  Name           = Stevens-Johnson syndrome   |
  Image          = |
  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 |
}}
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{{CMG}}
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==Overview==

'''Stevens-Johnson syndrome''' (SJS) is a life-threatening [[condition]] affecting the [[skin]], in which due to [[cell death]] the [[epidermis]] separates from the [[dermis]].  The syndrome is thought to be a [[hypersensitivity]] complex affecting the skin and the [[mucous membrane]]s.  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''').<ref>{{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}}</ref><ref>{{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}}</ref><ref>{{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 <!--Retrieved from CrossRef by DOI bot-->}}</ref><ref>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.</ref><ref name="pmid12164739">{{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 = 
}}</ref>

==Epidemiology==
SJS is a rare condition, with a reported incidence of around one case per million people 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]],<ref name="NZ">[http://www.medsafe.govt.nz/profs/Datasheet/d/DiflucanOnecap.htm Medsafe Data Sheet] March 8, 2005. Accessed April 26, 2007.</ref> [[valdecoxib]], [[penicillin]]s, [[barbiturate]]s, [[Sulfonamide (medicine)|sulfa]]s, [[phenytoin]], [[Modafinil]], [[lamotrigine]], [[nevirapine]], [[Ibuprofen]]<!--
  --><ref>{{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}}</ref>, [[ethosuximide]], [[carbamazepine]])<!--
  --><ref>{{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 <!--Retrieved from CrossRef by DOI bot-->}}</ref><!--
  --><ref>{{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}}</ref><!--
-->, malignancy ([[carcinoma]]s and [[lymphoma]]s), 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.<!--
  --><ref name="eMedicine">Stevens-Johnson Syndrome - {{eMedicine|emerg|555}}</ref>

Although Stevens Johnson Syndrome may be caused by viral infections or malignancies, severe allergic reactions to medication is the leading cause. Medications that have traditionally been known to lead to Stevens Johnson Syndrome, [[Erythema multiforme]], and [[Lyell's Syndrome]] ([[Toxic Epidermal Necrolysis]]) include; 

*[[sulfonamide]]s (antibiotics)
*[[penicillin]]'s (antibiotics)
*[[barbiturate]]s (sedatives)
*[[phenytoin]] - Dilantin (anticonvulsant)

==Treatment==
Discontinue all medications, 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.<!--
  --><ref name="eMedicine"/> 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.<ref>{{WhoNamedIt|synd|1501|Stevens-Johnson syndrome}}</ref><ref>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.</ref><ref>[http://dictionary.reference.com/browse/Stevens-Johnson%20syndrome Stevens-Johnson syndrome - Definitions from Dictionary.com<!-- Bot generated title -->]</ref>

==References==
{{Reflist|2}}

==External links==
* [http://www.sjsupport.org Stevens Johnson Syndrome Foundation] 
* [http://www.avimedi.net/en Association of victims of medicines]

{{Diseases of the skin and subcutaneous tissue}}

[[Category:Skin diseases]]




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