Erythema multiforme and Stevens-Johnson syndrome

Background

Stevens-Johnson syndrome is a blistering skin rash involving mucous membranes. There is a spectrum of involvement with different labels being given dependent on degree of skin involvement:¹

Feature	Erythema multiforme	SJS/TENS
Appearance	Raised target lesions	Flat target lesions progressing to vesicles and bullae
Fever	Rare	Common
Eye/mucosal involvement	Rare	Common
Precipitants	Infections, drugs, inflammatory bowel disease	Usually due to drugs
Extent of skin involvement		<10% SJS, >30% TENS

Precipitants may just be certain infections, but most frequently are medications (anti-epileptics, antibiotics, allopurinol)

Prognosis

There are various scoring systems which have been proposed (SCORTEN, ABCD-10), but they are limited by applicability in specific sub-populations.²,³

SCORTEN score	Mortality rate
0-1	3%
2	12%
3	35%
4	58%
>= 5	90%

Management

• Admit
• Stop all medications which could be causative, even if the association with particular agent is weak.
• IV fluids or nasogastric tube feeding as appropriate: for fluids and high protein diet. It may be necessary to suture in the NGT because of lack of intact skin to stick it to.
• The indications for steroids can best be described as tenuous. It is perfectly acceptable not to use them.
• Do NOT give prophylactic antibiotics. The illness itself can cause a disconcertingly high fever and if you have already given antibiotics to the patient, resistance patterns can quickly become problematic. Certainly keep looking for infection, and if the patient starts to deteriorate, consider septicaemia (particularly if the patient has been given corticosteroids).
• Analgesia – paracetamol 1 g 6 hourly may be adequate, but some patients require morphine. Paracetamol is described as causing the syndrome.
• Dressings are a source of considerable controversy and little data. Silver sulphadiazine is a sulpha containing product, and best avoided if there is any link to such agents as possible precipitants (e.g. cotrimoxazole, other sulphonamides).⁴
• Don’t forget about the eyes – steroid drops may be appropriate, but try to get ophthalmological advice.

Perspective – corticosteroid use in Stevens-Johnson syndrome.

There is little convincing evidence of a mortality benefit from corticosteroids use⁵. Where systematic reviews appear to show benefit, this is because of inclusion of observational studies, and even then the effect is unconvincing.⁶

DRESS

Drug rash with eosinophilia and systemic symptoms (often including elevated LFTs) is described after switching from one arene oxide anticonvulsant to another.⁷ The implication is that a patient who has reacted to phenytoin should not be given carbamazepine, and vice-versa. Even phenobarbitone has been implicated in cross-hypersensitivity⁸. Valproate is also described as causing SJS and is not necessarily a safe alternative.⁹

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1. Newkirk RE, Fomin DA, Braden MM. Erythema Multiforme Versus Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis: Subtle Difference in Presentation, Major Difference in Management. Mil Med. 2020 Sep 18;185(9-10):e1847-e1850. doi: 10.1093/milmed/usaa029. PMID: 32373930. ↩

2. Bastuji-Garin S, Fouchard N, Bertocchi M, et al. SCORTEN: a severity-of-illness score for toxic epidermal necrolysis. J Invest Dermatol. (2000) 115:149–53. doi: 10.1046/j.1523-1747.2000.00061.x ↩

3. Dobry AS, Himed S, Waters M, Kaffenberger BH. Scoring Assessments in Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis. Front Med (Lausanne). 2022 Jun 16;9:883121. doi: 10.3389/fmed.2022.883121. PMID: 35783656; PMCID: PMC9245022. ↩

4. Brüggen MC, Le ST, Walsh S, et al. Supportive care in the acute phase of Stevens-Johnson syndrome and toxic epidermal necrolysis: an international, multidisciplinary Delphi-based consensus. Br J Dermatol. 2021 Sep;185(3):616-626. doi: 10.1111/bjd.19893. Epub 2021 Apr 27. PMID: 33657677 ↩

5. Jacobsen A, Olabi B, Langley A, Beecker J, Mutter E, Shelley A, et al. Systemic interventions for treatment of Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), and SJS/TEN overlap syndrome. Cochrane Database Syst Rev. (2022) 3:CD013130. doi: 10.1002/14651858.CD013130.pub2 ↩

6. Zimmermann S, Sekula P, Venhoff M, et al. Systemic Immunomodulating Therapies for Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: A Systematic Review and Meta-analysis. JAMA Dermatol. 2017 Jun 1;153(6):514-522. doi: 10.1001/jamadermatol.2016.5668. PMID: 28329382; PMCID: PMC5817620. ↩

7. Kim CW, Choi GS, Yun CH, et al. Drug Hypersensitivity to previously tolerated phenytoin by carbamazepine-induced DRESS syndrome. J Korean Med Sci. 2006;21:768-72 ↩

8. Misra UK, Kalita J, Rathore C. Phenytoin and carbamazepine cross reactivity: report of a case and review of literature. Postgrad Med J. 2003;79:703-4 ↩

9. Naveen K, Arunkumar J, Hanumanthayya K, Pai V. Stevens-Johnson syndrome induced by sodium valproate monotherapy. Int J Crit Illn Inj Sci. 2012 Jan;2(1):44-5. doi: 10.4103/2229-5151.94904. PMID: 22624102; PMCID: PMC3354377. ↩

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