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Stevens-Johnson syndrome: causes, symptoms, diagnosis, treatment
Last reviewed: 05.07.2025
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Stevens-Johnson syndrome is an acute disease that has a severe course and causes blisters on the skin and mucous membranes. Stevens-Johnson syndrome occurs most often in young people, and men are more often affected than women.
What causes Stevens-Johnson syndrome?
The exact cause of Stevens-Johnson syndrome is unknown, but it is likely to be a perverted immune response. The most common and rapidly acting factor is hypersensitivity to drugs or viral infections. The main manifestation of Stevens-Johnson syndrome is acute vasculitis, which damages the skin and mucous membranes in all patients and the conjunctiva in 90% of patients. Stevens-Johnson syndrome is a self-limiting process: after the acute stage is over, most patients recover, and the functions of the damaged tissues are restored.
Symptoms of Stevens-Johnson Syndrome
Stevens-Johnson syndrome presents with fever, malaise, sore throat, possible cough and arthralgia, which last up to 2 weeks. Scabbed eyelids and self-limiting papillary conjunctivitis are the most common symptoms of Stevens-Johnson syndrome.
Severe membranous or pseudomembranous conjunctivitis with the development of limited conjunctival infarcts and the appearance of areas of fibrosis is less common.
After the acute phase of Stevens-Johnson syndrome, scarring does not occur.
Complications of Stevens-Johnson syndrome
- Symblepharon and keratinization.
- Lacrimation caused by blockage of the lacrimal points.
- "Dry" eye as a result of dysfunction of the lacrimal gland or blockage of the ducts.
- Secondary keratopathy due to cicatricial inversion of the eyelid, abnormal growth of eyelashes or keratinization of the conjunctiva.
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Treatment of Stevens-Johnson syndrome
Treatment of Stevens-Johnson syndrome involves the use of systemic corticosteroids. Acyclovir is prescribed if the cause of Stevens-Johnson syndrome is the herpes simplex virus.
Topical corticosteroids are started early and used throughout the disease to treat vasculitis and prevent the formation of areas of conjunctival necrosis.
A scleral ring, which consists of a large contact lens without a central zone, can be successfully used to prevent the development of symblepharon during the acute phase of the disease. Other treatments for Stevens-Johnson syndrome include topical retinoic acid to prevent the development of keratinization, tear substitutes, therapeutic contact lenses, as well as punctal occlusion and surgical correction of permanent deformities.