Stevens-Johnson syndrome

Medium urgency
-Moderately severe

Severe disorder of the skin and mucous membranes, in which the epidermis separates from the dermis forming blisters.

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

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

Diagnosis is made by clinical history and examination of 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 hospitalization. It has a recurrence risk of about 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 which produce painful sores


    Blisters in skinfolds


    Pain in mouth

Pre-hospital care recommendations

Take over-the-counter pain relievers or anti-inflammatories.
Maintain hydration of half a gallon per day. Avoid soft drinks and fruit juices with high sugar content.