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{{Stevens-Johnson syndrome}}
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==Overview==
==Overview ==
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>
Patients with Stevens-Johnson Syndrome usually have a history of recent drug use within the last 2 months or some other inciting trigger like infection or vaccination. It starts with a flu-like illness followed by skin eruptions that involve trunk and extremities mainly. Skin involvement in SJS is always <10 percent of the body surface. Blisters, erosions, vesicles and [[bullae]] are seen along with confluent redness and sloughing of skin. Mucosal lesions can be seen in 2 or more surfaces and can involve eyes, oral mucosa, nasopharynx or genitals commonly<ref name="pmid211627212">{{cite journal| author=Harr T, French LE| title=Toxic epidermal necrolysis and Stevens-Johnson syndrome. | journal=Orphanet J Rare Dis | year= 2010 | volume= 5 | issue=  | pages= 39 | pmid=21162721 | doi=10.1186/1750-1172-5-39 | pmc=3018455 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21162721  }}</ref>.
 
==History and Symptoms==
===History===
Patients with SJS may have a positive history of:
*Recent drug use
*Recent viral or bacterial infection
*Active malignancy
 
=== Symptoms ===
Symptoms usually start within 2 months of starting a drug or other inciting trigger.
* '''Prodome of flu like symptoms 1-3 days before skin eruptions'''. May include
** [[Fever]]
** [[Sore throat]]
** [[Chills]]
** [[Headache]]
** [[Malaise]]
** [[Cough]]
** [[Arthralgia|Arthralgias]]
* '''Skin lesions'''
** Involve less than 10 percent of body surface
** Start as red or reddish blue macules/ papules on trunk and extremities and later increase in size and coalesce
** Blisters/erosions/bullae and vesicles are seen
** Confluent redness with skin sloughing
** Nikolsky Sign is Positive- which is the detachment of superficial epidermis on gentle pressure or rubbing of skin
** [[Target lesions]] are not typically seen (as opposed to [[Erythema Multiforme]])
* '''Mucosal Lesions'''
** Can be seen before or after the skin eruptions
** Can involve 2 or more mucosal surfaces
** Seen in 90 percent of cases of SJS/TEN<ref name="pmid15875523">{{cite journal| author=Letko E, Papaliodis DN, Papaliodis GN, Daoud YJ, Ahmed AR, Foster CS| title=Stevens-Johnson syndrome and toxic epidermal necrolysis: a review of the literature. | journal=Ann Allergy Asthma Immunol | year= 2005 | volume= 94 | issue= 4 | pages= 419-36; quiz 436-8, 456 | pmid=15875523 | doi=10.1016/S1081-1206(10)61112-X | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15875523  }}</ref>
** Involved mucosal surfaces may include<ref name="pmid21162721">{{cite journal| author=Harr T, French LE| title=Toxic epidermal necrolysis and Stevens-Johnson syndrome. | journal=Orphanet J Rare Dis | year= 2010 | volume= 5 | issue=  | pages= 39 | pmid=21162721 | doi=10.1186/1750-1172-5-39 | pmc=3018455 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21162721  }}</ref>
*** Buccal mucosa: Painful erosions leading to [[Stomatitis]] and [[Mucositis]]
*** Ocular: Seen frequently and range from acute [[conjuctivitis]], eyelid edema and erythema, crusting, ocular discharge, Corneal erosion to corneal ulceration<ref name="pmid19243825">{{cite journal| author=Sotozono C, Ueta M, Koizumi N, Inatomi T, Shirakata Y, Ikezawa Z et al.| title=Diagnosis and treatment of Stevens-Johnson syndrome and toxic epidermal necrolysis with ocular complications. | journal=Ophthalmology | year= 2009 | volume= 116 | issue= 4 | pages= 685-90 | pmid=19243825 | doi=10.1016/j.ophtha.2008.12.048 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19243825  }}</ref>
*** Urogenital : Can cause [[Urethritis]], [[Vulvovaginitis]]
*** Nasopharynx
*** Tracheo-Bronchial and intestinal involvement is uncommon<ref name="pmid9470079">{{cite journal| author=Lebargy F, Wolkenstein P, Gisselbrecht M, Lange F, Fleury-Feith J, Delclaux C et al.| title=Pulmonary complications in toxic epidermal necrolysis: a prospective clinical study. | journal=Intensive Care Med | year= 1997 | volume= 23 | issue= 12 | pages= 1237-44 | pmid=9470079 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9470079  }}</ref>
 
==References==
==References==
{{reflist|2}}
{{Reflist|2}}
[[Category:Dermatology]]
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[[Category:Emergency medicine]]
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Latest revision as of 15:23, 15 September 2018

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Anila Hussain, MD [2]

Overview

Patients with Stevens-Johnson Syndrome usually have a history of recent drug use within the last 2 months or some other inciting trigger like infection or vaccination. It starts with a flu-like illness followed by skin eruptions that involve trunk and extremities mainly. Skin involvement in SJS is always <10 percent of the body surface. Blisters, erosions, vesicles and bullae are seen along with confluent redness and sloughing of skin. Mucosal lesions can be seen in 2 or more surfaces and can involve eyes, oral mucosa, nasopharynx or genitals commonly[1].

History and Symptoms

History

Patients with SJS may have a positive history of:

  • Recent drug use
  • Recent viral or bacterial infection
  • Active malignancy

Symptoms

Symptoms usually start within 2 months of starting a drug or other inciting trigger.

  • Prodome of flu like symptoms 1-3 days before skin eruptions. May include
  • Skin lesions
    • Involve less than 10 percent of body surface
    • Start as red or reddish blue macules/ papules on trunk and extremities and later increase in size and coalesce
    • Blisters/erosions/bullae and vesicles are seen
    • Confluent redness with skin sloughing
    • Nikolsky Sign is Positive- which is the detachment of superficial epidermis on gentle pressure or rubbing of skin
    • Target lesions are not typically seen (as opposed to Erythema Multiforme)
  • Mucosal Lesions
    • Can be seen before or after the skin eruptions
    • Can involve 2 or more mucosal surfaces
    • Seen in 90 percent of cases of SJS/TEN[2]
    • Involved mucosal surfaces may include[3]
      • Buccal mucosa: Painful erosions leading to Stomatitis and Mucositis
      • Ocular: Seen frequently and range from acute conjuctivitis, eyelid edema and erythema, crusting, ocular discharge, Corneal erosion to corneal ulceration[4]
      • Urogenital : Can cause Urethritis, Vulvovaginitis
      • Nasopharynx
      • Tracheo-Bronchial and intestinal involvement is uncommon[5]

References

  1. Harr T, French LE (2010). "Toxic epidermal necrolysis and Stevens-Johnson syndrome". Orphanet J Rare Dis. 5: 39. doi:10.1186/1750-1172-5-39. PMC 3018455. PMID 21162721.
  2. Letko E, Papaliodis DN, Papaliodis GN, Daoud YJ, Ahmed AR, Foster CS (2005). "Stevens-Johnson syndrome and toxic epidermal necrolysis: a review of the literature". Ann Allergy Asthma Immunol. 94 (4): 419–36, quiz 436-8, 456. doi:10.1016/S1081-1206(10)61112-X. PMID 15875523.
  3. Harr T, French LE (2010). "Toxic epidermal necrolysis and Stevens-Johnson syndrome". Orphanet J Rare Dis. 5: 39. doi:10.1186/1750-1172-5-39. PMC 3018455. PMID 21162721.
  4. Sotozono C, Ueta M, Koizumi N, Inatomi T, Shirakata Y, Ikezawa Z; et al. (2009). "Diagnosis and treatment of Stevens-Johnson syndrome and toxic epidermal necrolysis with ocular complications". Ophthalmology. 116 (4): 685–90. doi:10.1016/j.ophtha.2008.12.048. PMID 19243825.
  5. Lebargy F, Wolkenstein P, Gisselbrecht M, Lange F, Fleury-Feith J, Delclaux C; et al. (1997). "Pulmonary complications in toxic epidermal necrolysis: a prospective clinical study". Intensive Care Med. 23 (12): 1237–44. PMID 9470079.

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