Stevens-Johnson Syndrome (SJS)

What happens in SJS

SJS and TEN are the same disease at different severities. Immune cells (cytotoxic T-cells), triggered by a drug, release substances such as granulysin that kill skin cells widely. The epidermis separates from the layer beneath, like a severe burn. The linings of the mouth, eyes, genitals, and sometimes the airways and gut are also affected.

Causes

Medicines (most cases)

• allopurinol
• anticonvulsants: carbamazepine, lamotrigine, phenytoin, phenobarbital
• sulfonamide antibiotics such as trimethoprim-sulfamethoxazole
• nevirapine
• oxicam NSAIDs (such as meloxicam, piroxicam)
• sulfasalazine
• some other antibiotics and medicines

Infections

• Mycoplasma pneumoniae, especially in children. Some experts now call this pattern Mycoplasma-induced rash and mucositis. See mycoplasma pneumonia
• herpes simplex is more associated with erythema multiforme

Genetic risk

Certain HLA gene types raise risk dramatically with specific drugs. The FDA recommends screening people of Asian ancestry for HLA-B*15:02 before carbamazepine, and HLA-B 58:01 testing is recommended in some groups before allopurinol.

Other risk factors

• HIV infection
• previous SJS
• cancer, lupus
• older age

Symptoms

Early (1 to 3 days)

• fever
• sore throat and mouth
• burning, red eyes
• cough, body aches, fatigue

Then

• painful red or dusky purple spots, often starting on the face and chest, spreading quickly
• target-like spots with dark centers
• blisters that merge and burst, leaving raw, peeling areas
• painful sores and crusting of the lips, mouth and throat
• eye redness, discharge and pain
• genital and anal sores
• skin pain out of proportion to the rash

Diagnosis

• clinical recognition of the rash, mucosal involvement and drug history
• skin biopsy: shows full-thickness death of the epidermis, ruling out conditions such as staphylococcal scalded skin syndrome and autoimmune blistering diseases
• severity scoring (SCORTEN) to predict risk
• blood tests, infection screens and monitoring of organs

Treatment

Stop the trigger

Stopping the causative medicine immediately is the most important step and improves survival.

Supportive care

• admission to an intensive care or burn unit for extensive disease
• careful wound care with non-adherent dressings, avoiding adhesive tapes
• fluid and electrolyte replacement
• temperature control
• nutrition, often via a feeding tube
• pain control
• infection monitoring and treatment. Antibiotics are not given routinely
• prevention of blood clots

Eye, mouth and genital care

• daily ophthalmology review, lubricants, steroid drops and sometimes amniotic membrane grafts to protect vision
• mouth rinses and anesthetic washes
• genital care to prevent scarring and adhesions

Immune-modulating treatments

Cyclosporine, corticosteroids, IV immunoglobulin and TNF inhibitors such as etanercept have been used. Evidence is mixed, and choice varies between centers.

Complications

• skin infection and sepsis
• dehydration and kidney failure
• pneumonia and breathing failure
• long-term eye problems: dry eye, scarring, light sensitivity and vision loss
• skin color changes and scarring
• nail and hair loss
• genital and urethral scarring
• psychological trauma

Outlook

Mortality is roughly 10% for SJS and can be 30% or more for TEN, higher in older adults and those with other illnesses. Skin usually regrows within 2 to 3 weeks, but eye and other long-term effects can persist.

Prevention

• never take the causative drug, or chemically related drugs, again
• wear a medical alert bracelet
• inform all healthcare providers and pharmacies
• relatives may share genetic risk for specific drugs
• start lamotrigine at a low dose and increase slowly, as prescribed

Frequently asked questions

What is Stevens-Johnson syndrome?

It is a rare, serious reaction, usually to a medicine, in which the skin and mucous membranes blister and peel. It begins with flu-like symptoms followed by a painful spreading rash and sores in the mouth, eyes and genitals.

What medicines cause Stevens-Johnson syndrome?

Common triggers include allopurinol, carbamazepine, lamotrigine, phenytoin, sulfonamide antibiotics, nevirapine and oxicam NSAIDs. Reactions usually begin within the first 8 weeks of starting the drug.

What are the first signs of Stevens-Johnson syndrome?

Early signs are fever, sore throat, burning eyes, cough and body aches, followed within days by a painful red or purple rash that spreads and blisters. Mouth sores are common. Seek emergency care immediately.

What is the difference between SJS and TEN?

They are the same disease at different severities. SJS affects less than 10% of the body surface, toxic epidermal necrolysis (TEN) affects more than 30%, and overlap is in between. TEN has a higher death rate.

Can Stevens-Johnson syndrome happen again?

Yes, if the person takes the same drug or a closely related one again. Recurrences can be more severe. Permanently avoiding the trigger and recording it as an allergy is essential.