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Stevens-Johnson Syndrome

  • Definition

    Stevens-Johnson Syndrome is the most severe form of erythema multiforme and is characterized by ulcerated lesions on the skin and mucous membranes.

    It is a serious, sometimes fatal inflammatory disease. It is also known as erythema multiforme major.


    Erythema multiforme (EM) is acute inflammatory skin disease due to multiple causes. Erythema multiforme is divided into minor and major types based on the clinical findings.

    Approximately 90 percent of erythema multiforme minor follow outbreaks of herpes simplex.

    Erythema multiforme major (Stevens-Johnson syndrome) is marked by involvement of two or more mucosal surfaces (typically oral and conjunctival [eye]).


    The syndrome has been associated with a variety of infectious and pharmacologic agents. It is most often caused by drugs, especially sulfas, non-steroidal anti-inflammatory drugs (NSAIDs), and anticonvulsants such as phenytoin. Any drug is a potential cause.

    Coxsackievirus, echovirus, and most commonly, herpes simplex viruses as well as mycoplasma have precipitated the syndrome. Vaccines, such as those for tuberculosis and polio, have also been implicated. In approximately 50 percent of cases no cause can be identified.

    Stevens-Johnson syndrome can occur in all age groups.


    Typically, Stevens-Johnson syndrome affects the mucous membranes of the oral cavity, nostril, eyes, and both the anal and genital regions. It may or may not be associated with skin lesions elsewhere on the body. Oropharyngeal (mouth) lesions may be so intolerable as to prevent eating, and there may be recurring oral ulcers. Often, the skin lesions may look like a target-lesion (3 concentric zones of color change) or as bullae (bubble-like).

    A painful conjunctivitis may occur in the eye, frequently with a pus discharge, and can lead to corneal scarring and loss of vision. In addition to the mucous membrane lesions, fever and exhaustion are usual.

    Approximately one-third of patients have pulmonary involvement with cough and patchy infiltrates (material that has permeated into the tissues) on chest x-ray. In fatal cases, renal failure and pneumonia may occur.


    The diagnosis of Stevens-Johnson Syndrome is usually based on the clinical appearance and distribution of the skin and mucosal lesions. A skin biopsy may be performed.


    Every attempt should be made to identify a precipitating agent, and to remove it if possible. Antibiotics are appropriate if superinfection (a fresh infection added to one of the same nature already present) is suspected, or if bacterial disease, such as mycoplasma, is suspected to be the cause. Intensive supportive care is important in severe cases, including specialized care in a burn unit.

    Fluid replacement is often required, and meticulous oral hygiene is necessary to prevent superinfection. Examination by an ophthalmologist is recommended for patients with eye lesions so that precautions can be taken to avoid permanent eye damage. Oral and topical corticosteroids are often used.


    What is the cause?

    What treatment do you recommend to relieve symptoms?

    Will you be prescribing any medications? What are the side effects?

    What measure can be taken to avoid permanent eye damage? Has any permanent damage occurred?

    Can this occur again?