Stevens-Johnson syndrome

Medium urgency
-Moderately severe

A serious condition of the skin and mucous membranes in which the epidermis separates from the dermis to form blisters.

It is usually caused by a drug or infection, but in 50% of cases the cause is unknown. Other causes include infections, vaccines, hormonal changes, pregnancy, reactive arthritis and sarcoidosis. There are factors that increase the risk of Stevens-Johnson syndrome, such as having HIV, a weakened immune system, or a personal or family history of the syndrome.

It is characterised by the sudden onset of a rash with vesicles and blisters on the skin and mucous membranes, along with high fever, severe headache, sore throat and fatigue.

The diagnosis is made by taking a history and examining the lesions. Histological analysis of the sloughed skin shows a necrotic epithelium, which is a characteristic sign.

Pharmacological treatment is controversial and is based on intravenous immunoglobulin, corticosteroids and/or immunosuppressants.

The most common complications are dry eye syndrome, photophobia, blindness, sepsis, post-traumatic stress syndrome, respiratory failure, renal failure, arrhythmias and skin scarring.

It is a dermatological emergency requiring hospitalisation with a risk of recurrence of almost 40%.

Bibliographic references
  1. Kirchhof MG, Miliszewski MA, Sikora S, et al:Retrospective review of Stevens-Johnson syndrome/toxic epidermal necrolysis treatment comparing intravenous immunoglobulin with cyclosporine. J Am Acad Dermatol 71(5):941-947, 2014. doi: 10.1016/j.jaad.2014.07.016.
  2. Research in Stevens-Johnson syndrome/toxic epidermal necrolysis (TEN): a network of specialist TEN centres is needed to undertake effective clinical studies and therapeutic trials. Br J Dermatol. 2013 Dec;169(6):1177.
  3. The role of intravenous immunoglobulin in toxic epidermal necrolysis: a retrospective analysis of 64 patients managed in a specialized centre. Br J Dermatol. 2013 Dec;169(6):1304-9
  4. Diagnostic approach of erythroderma in the adult. Rev Med Inst Mex Seguro Soc. 2017 May-Jun;55(3):353-360.
  5. Severe cutaneous adverse reactions: acute generalized exanthematous pustulosis, toxic epidermal necrolysis and Stevens-Johnson syndrome. Harr T, French LE. Med Clin North Am. 2010 Jul; 94(4):727-42, x.
  6. Retrospective review of Stevens-Johnson syndrome/toxic epidermal necrolysis treatment comparing intravenous immunoglobulin with cyclosporine. J Am Acad Dermatol. 2014 Nov;71(5):941-7
  7. Del Pozzo-Magana BR, Lazo-Langner A, Carleton B, Castro-Pastrana LI, Rieder MJ. A systematic review of treatment of drug-induced Stevens-Johnson syndrome and toxic epidermal necrolysis in children. J Popul Ther Clin Pharmacol. 2011;18:e121-33
  8. French LE. Toxic epidermal necrolysis and Stevens Johnson syndrome: our current understanding. Allergol Int. 2006 Mar;55(1):9-16
  9. Molgó M, Carreño N, Hoyos-Bachiloglu R, Andresen M, González S. Use of intravenous immunoglobulin for the treatment of toxic epidermal necrolysis and Stevens-Johnson/toxic epidermal necrolysis overlap syndrome. Review of 15 cases. Rev Med Chil. 2009 Mar;137(3):383-9. Epub 2009 Jun 15
  10. Pasternack MS, Swartz MN. Cellulitis, necrotizing fasciitis, and subcutaneous tissue infections. In: Mandell GL, Bennett JE, Dolin R, eds. Principles and Practice of Infectious Diseases. 7th ed. Philadelphia, Pa: Elsevier Churchill Livingstone; 2009:chap 90.
  11. Schumann-Gable N. Dermatology. In: Custer JW, Rau RE, eds. The Harriet Lane Handbook. 18th ed. Philadelphia, Pa: Elsevier Mosby; 2009:chap 8.
  12. Wetter DA, Camilleri MJ.Clinical, etiologic, and histopathologic features of Stevens-Johnson syndrome during an 8-year period at Mayo Clinic. Mayo Clin Proc. 2010 Feb;85(2):131-8.
Author
Dr. Abel Andrés Orelogio
Copyright
© TeckelMedical 2026

Symptoms

    Blisters that flake off


    Painful ulcer


    Genital vesicles that turn into painful ulcers


    Blisters in skinfolds


    Pain in mouth

Pre-hospital care recommendations

Consume over-the-counter pain relievers or anti-inflammatories.
Maintain hydration of 2L per day. Avoid soft drinks and fruit juices with high sugar content.