Stevens-Johnson syndrome overview: Difference between revisions
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==Overview== | ==Overview== | ||
'''Stevens-Johnson syndrome''' 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. | '''Stevens-Johnson syndrome''' 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. | ||
==Historical Perspective== | |||
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>[http://dictionary.reference.com/browse/Stevens-Johnson%20syndrome Stevens-Johnson syndrome - Definitions from Dictionary.com</ref> | |||
==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> | |||
==Pathophysiology== | |||
SJS, like [[toxic epidermal necrolysis]] and [[erythema multiforme]], are characterized by confluent epidermal [[necrosis]] with minimal associated inflammation. The acuity is apparent from the (normal) basket weave-like pattern of the [[stratum corneum]]. | |||
==Epidemiology and Demographics== | |||
SJS is a rare condition, with a reported incidence of around one case per 100,000 people per year. | |||
==Natural History, Complications and 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. | |||
==Diagnosis== | |||
===History and Symptoms=== | |||
Stevens–Johnson syndrome (SJS) usually begins with fever, sore throat, and fatigue, which is misdiagnosed and usually treated with antibiotics. Ulcers and other lesions begin to appear in the mucous membranes, almost always in the mouth and lips but also in the genital and anal regions. Those in the mouth are usually extremely painful and reduce the patient's ability to eat or drink. [[Conjunctivitis]] of the eyes occurs in about 30% of children who develop SJS. A rash of round lesions about an inch across arises on the face, trunk, arms and legs, and soles of the feet, but usually not the scalp.<ref name=Tigchelaar>{{cite journal |author=Tigchelaar H | coauthors = Kannikeswaran N ; Kamat D |title=Stevens–Johnson Syndrome: An Intriguing Diagnosis |journal=Consultant for Pediatricians|month=December | year = 2008 |url=http://www.pediatricsconsultantlive.com/display/article/1803329/1403936 }}</ref> | |||
==References== | ==References== | ||
Revision as of 17:45, 11 February 2013
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Stevens-Johnson syndrome 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 membranes. Although the majority of cases are idiopathic, the main class of known causes is medications, followed by infections and (rarely) cancers.
Historical Perspective
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.[1][2]
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).[3][4][5][6][7]
Pathophysiology
SJS, like toxic epidermal necrolysis and erythema multiforme, are characterized by confluent epidermal necrosis with minimal associated inflammation. The acuity is apparent from the (normal) basket weave-like pattern of the stratum corneum.
Epidemiology and Demographics
SJS is a rare condition, with a reported incidence of around one case per 100,000 people per year.
Natural History, Complications and 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.
Diagnosis
History and Symptoms
Stevens–Johnson syndrome (SJS) usually begins with fever, sore throat, and fatigue, which is misdiagnosed and usually treated with antibiotics. Ulcers and other lesions begin to appear in the mucous membranes, almost always in the mouth and lips but also in the genital and anal regions. Those in the mouth are usually extremely painful and reduce the patient's ability to eat or drink. Conjunctivitis of the eyes occurs in about 30% of children who develop SJS. A rash of round lesions about an inch across arises on the face, trunk, arms and legs, and soles of the feet, but usually not the scalp.[8]
References
- ↑ Template:WhoNamedIt
- ↑ [http://dictionary.reference.com/browse/Stevens-Johnson%20syndrome Stevens-Johnson syndrome - Definitions from Dictionary.com
- ↑ Carrozzo M, Togliatto M, Gandolfo S (1999). "[Erythema multiforme. A heterogeneous pathologic phenotype]". Minerva Stomatol. 48 (5): 217–26. PMID 10434539.
- ↑ Farthing P, Bagan J, Scully C (2005). "Mucosal disease series. Number IV. Erythema multiforme". Oral Dis. 11 (5): 261–7. PMID 16120111.
- ↑ Bastuji-Garin S, Rzany B, Stern RS, Shear NH, Naldi L, Roujeau JC (1993). "Clinical classification of cases of toxic epidermal necrolysis, Stevens-Johnson syndrome, and erythema multiforme". Archives of dermatology. 129 (1): 92–6. doi:10.1001/archderm.129.1.92. PMID 8420497.
- ↑ Ogundele O, Silverberg MA. Erythema Multiforme. eMedicine.com. URL: http://www.emedicine.com/emerg/topic173.htm. Accessed on: May 6, 2007.
- ↑ Auquier-Dunant A, Mockenhaupt M, Naldi L, Correia O, Schröder W, Roujeau JC (2002). "Correlations between clinical patterns and causes of erythema multiforme majus, Stevens-Johnson syndrome, and toxic epidermal necrolysis: results of an international prospective study". Arch Dermatol. 138 (8): 1019–24. PMID 12164739. Unknown parameter
|month=ignored (help) - ↑ Tigchelaar H (2008). "Stevens–Johnson Syndrome: An Intriguing Diagnosis". Consultant for Pediatricians. Unknown parameter
|coauthors=ignored (help); Unknown parameter|month=ignored (help)