Stevens-Johnson syndrome (aka Erythema multiforme major) is a rare and potentially life threatening inflammatory disease in which the skin and mucous membranes react severely to a particular type of medication or infection. Stevens-Johnson syndrome starts with flu-like symptoms and is followed by painful red or purple colored rashes that spread and form blisters across the skin, eventually causing the top layer to shed and die.
Causes
Stevens-Johnson Syndrome has most often been caused by drugs, especially sulfas, non-steroidal anti-inflammatory drugs (NSAIDs), and anticonvulsants such as phenytoin. Any drug, however, is a potential cause.
Coxsackievirus, echovirus, and most commonly, herpes simplex viruses as well as mycoplasma have been known to trigger the syndrome. Vaccines, such as those used to treat tuberculosis and polio, have also been linked to the disease. In about 50 percent of cases, no causes was identified.
Victims of Stevens-Johnson generally tend to be between the second and fourth decades of their lives. However, the disease has affect children as young as three months and any age group is susceptible.
Symptoms
Stevens-Johnson syndrome typically 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 can be especially intolerable and can hinder eating. There may also 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
Diagnosis of Stevens-Johnson Syndrome is based on the clinical appearance and distribution of the skin and mucosal lesions. Skin biopsies are often performed to detect Stevens-Johnson syndrome.
Treatment
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.
Sources:
http://www.healthscout.com/ency/68/698/main.html%23SymptomsofStevens-JohnsonSyndrome
