What is Stevens-Johnson Syndrome (SJS), and what information should I provide to patients about it?

Kristina Blevins,RN, and Bridget Parsh, EdD, CNS, RN reply-SJS is a severe mucocutaneous reaction, most commonly triggered by medications, characterized by extensive necrosis and detachment of the epidermis.1 Mucous membranes are affected in over 90% of patients, usually at two or more distinct sites (ocular, oral, and genital).1

SJS is not to be confused with toxic epidermal necrolysis (TEN), though they are considered a disease continuum with SJS being less severe based upon the percentage of total body surface area (TBSA) affected by blisters and erosions.2-4 TEN involves more than 30% of the TBSA, whereas SJS involves up to 10% of TBSA.2,3,5

SJS can present in any individual, although it is more common in children, older adults, and females.3,5 There are between 1 and 2 million cases of SJS per year worldwide with a mortality rate of about 5%. Sepsis is often the major cause of death.4 Older adults have higher mortality due to factors such as multiple comorbidities, polypharmacy, concomitant radiotherapy and antiepileptic drugs (AEDs), and genetic susceptibility.5,6,9

Immunosuppression also plays a role in SJS. In those with HIV, the risk of developing SJS is 1,000 times greater.3,7 Those with systemic lupus erythematosus, active malignancy, and a history of bone marrow transplantation are also at a greater risk for developing SJS.5

Most cases of SJS are related to medications such as antibiotics like sulfonamides and beta-lactams, nonsteroidal anti-inflammatory drugs (oxicam-type), allopurinol, and AEDs such as phenytoin, phenobarbital, and carbamazepine.1,3,5-9

Signs and symptoms of SJS usually occur 4 to 28 days after starting a new drug.9 Fever (often higher than 102[degrees] F or 39[degrees] C), flulike illness, itching or burning eyes, joint pain, or cough, often precede the development of mucocutaneous lesions by 1 to 3 days.9,10 Skin signs and symptoms include patches of erythematous and painful skin; ecchymotic areas with blisters; blisters or peeling skin on the chest, face, palms of hands, or soles of feet; lesions, edema, and crusting on the mucous membranes of the mouth, eyes, vagina, and penis.

Long-term complications include scarring and dyspigmentation of skin.7 With the loss of skin integrity, secondary bacterial infections, as well as electrolyte imbalances and loss of thermoregulation, can occur.9 Long-term pulmonary complications include chronic bronchitis/bronchiolitis with obstructive changes (including bronchiolitis obliterans and bronchiolitis obliterans organizing pneumonia), bronchiectasis, and obstructive disorders.

Management of patients with suspected SJS includes early recognition, immediate cessation of the suspected trigger drug, and hospital admission. Depending upon the extent of skin involvement and the presence of comorbidities, the patient may need to be transferred to an ICU or burn unit. Supportive care is the mainstay of treatment and includes wound care, fluid and electrolyte management, nutritional support, ocular care, temperature management, pain control, and monitoring or treatment of superinfections.

Nurses should obtain a patient's detailed health history including signs and symptoms suggestive of SJS and any known risk factors.

Document all medications taken over the previous 2 months including the dose, date when drugs were started, and the date drugs were discontinued, as applicable.11 In addition, note any previous history of drug allergies and reaction.11

Document the reaction in the patient's medical record and teach the patient the names of medications that may cause SJS (See Drugs associated with SJS risk).11 Encourage patients to avoid over-the-counter drugs with unknown or unclear ingredients and to wear a medical alert ID.

Teach patients the signs and symptoms of SJS, emphasize the importance of notifying their healthcare provider should any occur.7,9 Early recognition and referral to the surgical or burn team is key to ensuring the best patient outcomes.

Drugs associated with SJS risk11

Allopurinol

Carbamazepine

Lamotrigine

Nevirapine

Oxicam NSAIDs

Phenobarbital

Phenytoin

Sulfamethoxazole

Sulfasalazine

Note: This list is not exhaustive.

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