Stevens-Johnson syndrome: symptoms, what is it?

Stevens-Johnson Syndrome (SSJ) is a rare and incurable skin disease. It causes in particular fever, necrosis and plaques on the skin and mucous membranes. The disease is essentially caused by certain drugs, Treatment is symptomatic. What is Stevens-Johnson Syndrome? What are symptoms ? How to diagnose this skin disease?

Definition: What is Stevens-Johnson Syndrome?

Stevens-Johnson syndrome is a rare skin disease which appears in a brutal way and which can lead to death (about 15%, 20% of cases). Less extensive form of Lyell’s syndrome (defined by a detachment affecting more than 30% of the skin surface vs less than 10% for Stevens Johnson syndrome), Stevens-Johnson syndrome is an incurable disease to this day. The proposed treatments aim in particular to reduce the risk of infection.

What are the symptoms of Stevens-Johnson Syndrome?

This syndrome is manifested by fever, itchy eyes or mouth pain and the formation of more or less large plaques on the skin and mucous membranes. Sometimes the lesions reach the internal organs (lungs, digestive tract). The most serious sequelae are ocular sequelae which can lead to blindness.

What causes Stevens-Johnson Syndrome?

The disease is essentially caused by certain drugsin particular sulfonamides, allopurinol or anticonvulsants. In its list of drugs to avoid, the medical journal Prescribe cites the following drugs as being able to expose to Stevens-Johnson syndrome:

• agomelatine
• duloxetine (Cymbalta® or other),
• etifoxine (Stresam®)
• bupropione (Zyban®),
• meloxicam (Mobic® or other), piroxicam (Feldene® or other) and tenoxicam (Tilcotil®) in general expose to an increase in digestive and cutaneous disorders (including Stevens-Johnson syndromes and Lyell syndromes,
• ambroxol (Muxol® or other) and bromhexine (Bisolvon®).

Infectious causes have also been reported. Sometimes the cause is idiopathic, that is, unknown. “This disease has been considered the most severe allergic disease because it results from an uncontrolled reaction of the immune system which will develop specific T cells against the ingested drug. These will activate very strongly and cause a epidermal necrolysis (detachment of the skin), by necrosis and/or massive death of epidermal cells. There are genetic risk factorsespecially in Asian patients“, develops Dr. Benoit Ben Said.

The diagnosis is clinical. Stevens-Johnson syndrome can be suspected at the appearance of the skin and mucous membranes reached. A biopsy can be performed to confirm necrolysis of the epidermis. In some cases, a allergological check-up with skin tests may be offered. “It exists two certified reference centers in France: one in Lyon and one in Paris at the Henri Mondor hospital. There are also competence centers that can also take care of Stevens-Johnson syndrome. Management in an expert center improves the prognosis“, argues the dermatologist and venerologist.

What is the treatment for Stevens-Johnson Syndrome?

The treatment is symptomatic. Stevens-Johnson syndrome requires a care in a reference center or failing that, in a burns center or an intensive care unit. Symptomatic treatment aimed in particular at limit the risk of infection is administered to the patient, together with intravenous fluids to compensate for fluid losses associated with skin detachments. “The offending drug should be stopped as soon as possible to decrease the risk of mortality and the family of drugs should be avoided for life. So far, few clinical trials have been conducted. In Lyon, a therapeutic trial is currently underway, the results will be known within three years. Another trial will be conducted in Paris in the coming months“, says the specialist.

Thanks to Dr. Benoit Ben Said, dermatologist and venerologist, coordinator of the reference center for toxic bullous dermatoses and severe drug eruption at the Hospices Civils de Lyon.