Stevens-Johnson syndrome natural history, complications and prognosis

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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. Appropraite 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 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

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.

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