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Health Dictionary

Reliable, easy-to-read patient information on common medical conditions — written in plain language, free of jargon, and grounded in current evidence. Browse the most frequent diagnoses, understand what they mean for everyday life, and learn which steps and questions help you have a focused conversation with your doctor.

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Stevens-Johnson syndrome natural history, complications and prognosis

Introduction

Overview

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, 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

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

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