Definition of Stevens-Johnson Syndrome

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.
Description of Stevens-Johnson Syndrome
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]).
Causes and Risk Factors of Stevens-Johnson Syndrome
The syndrome has been associated with a variety of infectious and pharmacologic
agents. It is most often caused by drugs, especially sulfas, non-steroidal antiinflammatory 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.
Symptoms of Stevens-Johnson Syndrome
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.

Diagnosis of Stevens-Johnson Syndrome

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.
Treatment of Stevens-Johnson Syndrome
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.
Questions To Ask Your Doctor About Stevens-Johnson Syndrome
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?