Stevens-Johnson syndrome natural history, complications and prognosis: Difference between revisions

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{{Stevens-Johnson syndrome}}
{{Stevens-Johnson syndrome}}


{{CMG}}; {{AE}}  
{{CMG}}; {{AE}} {{AHS}}


==Overview==
==Overview==
If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
*The symptoms of SJS usually start within 2 months of starting a drug or other inciting trigger with symptoms such 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 later with confluent redness with skin sloughing. Most lesions heal rapidly within 1-3 weeks unless secondary bacterial infection of lesions occurs. Recovery can take longer, although it usually depends on the severity of symptoms and body surface area involved. Mortality rate is 1-5 percent in SJS and in TEN is 25-35 percent<ref name="pmid211627212" />


OR
Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
OR
Prognosis is generally excellent/good/poor, and the 1/5/10-year mortality/survival rate of patients with [disease name] is approximately [#]%.
==Natural History, Complications, and Prognosis==
==Natural History, Complications, and Prognosis==


===Natural History===
===Natural History===
*The symptoms of (disease name) usually develop in the first/ second/ third decade of life, and start with symptoms such as ___.  
*The symptoms of SJS usually start within 2 months of starting a drug or other inciting trigger with symptoms such 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 later with confluent redness with skin sloughing.  
*The symptoms of (disease name) typically develop ___ years after exposure to ___.  
*Most lesions heal rapidly within 1-3 weeks unless secondary bacterial infection of lesions occurs.  
*If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
*Recovery can take longer, although it usually depends on the severity of symptoms and body surface area involved


===Complications===
===Complications===
*Common complications of [disease name] include:
*Common complications of SJS/TEN include:
**[Complication 1]
**Bacterial Infections leading to sepsis - more common in age>40, WBC>10,00, BSA more than or equal to 30 percent<ref name="pmid20075702">{{cite journal| author=de Prost N, Ingen-Housz-Oro S, Duong Ta, Valeyrie-Allanore L, Legrand P, Wolkenstein P et al.| title=Bacteremia in Stevens-Johnson syndrome and toxic epidermal necrolysis: epidemiology, risk factors, and predictive value of skin cultures. | journal=Medicine (Baltimore) | year= 2010 | volume= 89 | issue= 1 | pages= 28-36 | pmid=20075702 | doi=10.1097/MD.0b013e3181ca4290 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20075702  }}</ref>
**[Complication 2]
**Opthalmologic - Ranging from pain, [[photophobia]], [[conjuctivitis]] to [[keratitis]] and [[endopthalmitis]]. Appropriate treatment of acute ocular complications can prevent long term complications<ref name="pmid270182342">{{cite journal| author=Catt CJ, Hamilton GM, Fish J, Mireskandari K, Ali A| title=Ocular Manifestations of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis in Children. | journal=Am J Ophthalmol | year= 2016 | volume= 166 | issue=  | pages= 68-75 | pmid=27018234 | doi=10.1016/j.ajo.2016.03.020 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27018234  }}</ref>
**[Complication 3]
**Pulmonary - [[Pnemonia]], [[Interstitial pneumonitis]], [[Acute respiratory distress syndrome|ARDS]] (25 percent of patients have been reported to require mechanical ventilation)<ref name="pmid23989174">{{cite journal| author=de Prost N, Mekontso-Dessap A, Valeyrie-Allanore L, Van Nhieu JT, Duong TA, Chosidow O et al.| title=Acute respiratory failure in patients with toxic epidermal necrolysis: clinical features and factors associated with mechanical ventilation. | journal=Crit Care Med | year= 2014 | volume= 42 | issue= 1 | pages= 118-28 | pmid=23989174 | doi=10.1097/CCM.0b013e31829eb94f | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23989174  }}</ref>
**Skin - Scarring or hypertrophic changes
 
===Prognosis<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>===
*Prognosis and severity of SJS/TEN should be determined immediately after diagnosis to decide upon the appropriate medical management and setting.
*SCORTEN Severity of illness score may be used to determine the severity and prognosis
{| class="wikitable"
|+'''SCORTEN SEVERITY OF ILLNESS SCORE'''<ref name="pmid10951229">{{cite journal| author=Bastuji-Garin S, Fouchard N, Bertocchi M, Roujeau JC, Revuz J, Wolkenstein P| title=SCORTEN: a severity-of-illness score for toxic epidermal necrolysis. | journal=J Invest Dermatol | year= 2000 | volume= 115 | issue= 2 | pages= 149-53 | pmid=10951229 | doi=10.1046/j.1523-1747.2000.00061.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10951229  }}</ref>
!'''SCORTEN Parameter'''
!'''Individual score'''
!'''SCORTEN (sum of individual scores)'''
!'''Predicted mortality (%)'''
|-
|Age > 40 years
|Yes = 1, No = 0
|0-1
|3.2
|-
|Malignancy
|Yes = 1, No = 0
|2
| colspan="1" rowspan="1" |12.1
|-
|Tachycardia (> 120/min)
|Yes = 1, No = 0
|3
|35.8
|-
|Initial surface of epidermal detachment >10%
|Yes = 1, No = 0
|4
|58.3
|-
|Serum urea >10 mmol/l
|Yes = 1, No = 0
|>5
|90
|-
|Serum glucose >14 mmol/l
|Yes = 1, No = 0
|
|
|-
|Bicarbonate >20 mmol/l
|Yes = 1, No = 0
|
|
|}
*Patients with SCORTEN score of 0-1 with limited skin involvement can be manged in non-specialized wards<ref>Valeyrie-Allanore L, Roujeau J-C. Epidermal necrolysis (Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis). In: Fitzpatrick’s Dermatology in General Medicine, 8th Edition, Goldsmith LA, Katz SI, Gilchrest BA, Paller AS. (Eds), Mcgraw-Hill, 2012.</ref>
*Patients with the SCORTEN score of 3 or more should be treated in an ICU.
*Depending on the extent of the skin sloughing/ Body surface are involved at the time of diagnosis, the prognosis may vary. However, the prognosis is generally regarded as good
*Mortality rate is 1-5 percent in SJS and in TEN is 25-35 percent<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>
*Mortality rate can be higher in elderly and those with extensive skin involvement


===Prognosis===
=== Negative Prognostic Factors: ===
*Prognosis is generally excellent/good/poor, and the 1/5/10-year mortality/survival rate of patients with [disease name] is approximately [--]%.
May include<ref>Schulz J, Sheridan RL, Ryan CM, et al. A 10-year experience with toxic epidermal necrolysis. ''J Burn Care Rehabil'' 2000. 21: 199-204</ref><ref>Namdar T, von Wild T, Siemers F, et al. Does hypernatremia impact mortality in toxic epidermal necrolysis? ''Ger Med Sci'' 2010; 8:doc30</ref><ref>Westly ED, Wechsler HL. Toxic epidermal necrolysis: granulocytic leucopenia as a prognostic indicator. ''Arch Dermatol'' 1984; 120:721-726</ref>:
*Depending on the extent of the [tumor/disease progression] at the time of diagnosis, the prognosis may vary. However, the prognosis is generally regarded as poor/good/excellent.
* [[Hypernatremia]]
*The presence of [characteristic of disease] is associated with a particularly [good/poor] prognosis among patients with [disease/malignancy].
* [[Neutropenia]]
*[Subtype of disease/malignancy] is associated with the most favorable prognosis.
* [[Thrombocytopenia]]
*The prognosis varies with the [characteristic] of tumor; [subtype of disease/malignancy] have the most favorable prognosis.
* Increased BUN


==References==
==References==

Latest revision as of 15:21, 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

  • The symptoms of SJS usually start within 2 months of starting a drug or other inciting trigger with symptoms such 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 later with confluent redness with skin sloughing. Most lesions heal rapidly within 1-3 weeks unless secondary bacterial infection of lesions occurs. Recovery can take longer, although it usually depends on the severity of symptoms and body surface area involved. Mortality rate is 1-5 percent in SJS and in TEN is 25-35 percent[1]

Natural History, Complications, and Prognosis

Natural History

  • The symptoms of SJS usually start within 2 months of starting a drug or other inciting trigger with symptoms such 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 later with confluent redness with skin sloughing.
  • Most lesions heal rapidly within 1-3 weeks unless secondary bacterial infection of lesions occurs.
  • Recovery can take longer, although it usually depends on the severity of symptoms and body surface area involved

Complications

  • Common complications of SJS/TEN include:
    • Bacterial Infections leading to sepsis - more common in age>40, WBC>10,00, BSA more than or equal to 30 percent[2]
    • Opthalmologic - Ranging from pain, photophobia, conjuctivitis to keratitis and endopthalmitis. Appropriate treatment of acute ocular complications can prevent long term complications[3]
    • Pulmonary - Pnemonia, Interstitial pneumonitis, ARDS (25 percent of patients have been reported to require mechanical ventilation)[4]
    • Skin - Scarring or hypertrophic changes

Prognosis[5]

  • Prognosis and severity of SJS/TEN should be determined immediately after diagnosis to decide upon the appropriate medical management and setting.
  • SCORTEN Severity of illness score may be used to determine the severity and prognosis
SCORTEN SEVERITY OF ILLNESS SCORE[6]
SCORTEN Parameter Individual score SCORTEN (sum of individual scores) Predicted mortality (%)
Age > 40 years Yes = 1, No = 0 0-1 3.2
Malignancy Yes = 1, No = 0 2 12.1
Tachycardia (> 120/min) Yes = 1, No = 0 3 35.8
Initial surface of epidermal detachment >10% Yes = 1, No = 0 4 58.3
Serum urea >10 mmol/l Yes = 1, No = 0 >5 90
Serum glucose >14 mmol/l Yes = 1, No = 0
Bicarbonate >20 mmol/l Yes = 1, No = 0
  • Patients with SCORTEN score of 0-1 with limited skin involvement can be manged in non-specialized wards[7]
  • Patients with the SCORTEN score of 3 or more should be treated in an ICU.
  • Depending on the extent of the skin sloughing/ Body surface are involved at the time of diagnosis, the prognosis may vary. However, the prognosis is generally regarded as good
  • Mortality rate is 1-5 percent in SJS and in TEN is 25-35 percent[1]
  • Mortality rate can be higher in elderly and those with extensive skin involvement

Negative Prognostic Factors:

May include[8][9][10]:

References

  1. ↑ 1.0 1.1 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. ↑ de Prost N, Ingen-Housz-Oro S, Duong Ta, Valeyrie-Allanore L, Legrand P, Wolkenstein P; et al. (2010). "Bacteremia in Stevens-Johnson syndrome and toxic epidermal necrolysis: epidemiology, risk factors, and predictive value of skin cultures". Medicine (Baltimore). 89 (1): 28–36. doi:10.1097/MD.0b013e3181ca4290. PMID 20075702.
  3. ↑ Catt CJ, Hamilton GM, Fish J, Mireskandari K, Ali A (2016). "Ocular Manifestations of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis in Children". Am J Ophthalmol. 166: 68–75. doi:10.1016/j.ajo.2016.03.020. PMID 27018234.
  4. ↑ de Prost N, Mekontso-Dessap A, Valeyrie-Allanore L, Van Nhieu JT, Duong TA, Chosidow O; et al. (2014). "Acute respiratory failure in patients with toxic epidermal necrolysis: clinical features and factors associated with mechanical ventilation". Crit Care Med. 42 (1): 118–28. doi:10.1097/CCM.0b013e31829eb94f. PMID 23989174.
  5. ↑ 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.
  6. ↑ Bastuji-Garin S, Fouchard N, Bertocchi M, Roujeau JC, Revuz J, Wolkenstein P (2000). "SCORTEN: a severity-of-illness score for toxic epidermal necrolysis". J Invest Dermatol. 115 (2): 149–53. doi:10.1046/j.1523-1747.2000.00061.x. PMID 10951229.
  7. ↑ Valeyrie-Allanore L, Roujeau J-C. Epidermal necrolysis (Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis). In: Fitzpatrick’s Dermatology in General Medicine, 8th Edition, Goldsmith LA, Katz SI, Gilchrest BA, Paller AS. (Eds), Mcgraw-Hill, 2012.
  8. ↑ Schulz J, Sheridan RL, Ryan CM, et al. A 10-year experience with toxic epidermal necrolysis. J Burn Care Rehabil 2000. 21: 199-204
  9. ↑ Namdar T, von Wild T, Siemers F, et al. Does hypernatremia impact mortality in toxic epidermal necrolysis? Ger Med Sci 2010; 8:doc30
  10. ↑ Westly ED, Wechsler HL. Toxic epidermal necrolysis: granulocytic leucopenia as a prognostic indicator. Arch Dermatol 1984; 120:721-726

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