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. 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 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:
- Hypernatremia
- Neutropenia
- Thrombocytopenia
- Increased BUN
References
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ Schulz J, Sheridan RL, Ryan CM, et al. A 10-year experience with toxic epidermal necrolysis. J Burn Care Rehabil 2000. 21: 199-204
- ↑ Namdar T, von Wild T, Siemers F, et al. Does hypernatremia impact mortality in toxic epidermal necrolysis? Ger Med Sci 2010; 8:doc30
- ↑ Westly ED, Wechsler HL. Toxic epidermal necrolysis: granulocytic leucopenia as a prognostic indicator. Arch Dermatol 1984; 120:721-726