Steven-Johnson syndrome

Overview

Stevens-Johnson syndrome (SJS) is a severe skin disorder that typically necessitates hospitalization. This condition causes rashes, blisters, and peeling skin, also affecting mucus membranes like the eyes, mouth, and genitalia. It is often triggered by a medication reaction, and healing process may take weeks or months.

Some people consider SJS and toxic epidermal necrolysis (TEN) as different diseases, while others view them as the same disease with SJS being less severe. In SJS, less than 10% of the skin peels, while in TEN, over 30% of the skin is affected. However, both conditions can be life-threatening.

SJS is a severe condition that necessitates immediate medical attention and typically requires admittance to a hospital for proper treatment. If the condition was caused by a medication, permanently avoiding that drug and similar ones may help. Treatment for SJS usually focuses on treating the underlying cause, tending to wounds, managing pain, and reducing complications as the skin heals.

Symptoms

One to three days before a rash develops, early signs of Stevens-Johnson syndrome may include fever, a sore mouth and throat, fatigue, and burning eyes.

Common symptoms of Stevens-Johnson syndrome include:

• Pain on the skin
• Painful throat and mouth
• Cough
• Spreading red or purple rashes on the body
• Ulcers and blisters on the skin, as well as on the mucous membranes of the eyes, mouth, throat, genitalia, and anus
• Skin shedding that occurs days after blister formation
• Painful urination as a result of mucus membrane blisters

If any symptoms persist, seeking prompt medical attention is advisable for accurate diagnosis and treatment. Medications can potentially trigger this condition, even after discontinuation, with symptoms possibly resurfacing up to two weeks later. Recurrence is possible, typically with heightened severity upon subsequent occurrences.

Causes

Stevens-Johnson syndrome is an uncommon and unpredictable condition. If a drug triggers it, symptoms appear one to three weeks after starting the medication, beginning with flu-like symptoms, and followed by a rash and skin peeling. In severe cases, hair and nails may also be lost.

Stevens-Johnson syndrome can be caused by an allergic reaction to medication, infections like pneumonia and herpes, vaccinations, graft-versus-host disease, or sometimes no known cause.

Medications associated with Stevens-Johnson Syndrome encompass anti-gout drugs such as allopurinol, anticonvulsants, antipsychotics, sulfonamide antibiotics, nevirapine, and pain relievers like acetaminophen, ibuprofen, and naproxen sodium.

Risk factors

Factors that increases the risk of SJS include:

• Having SJS from a medication before, and taking that drug again
• Having a close relative or immediate family member with SJS
• Having certain genetic factors, especially if taking drugs for seizures, gout, or mental illness
• Having HIV, which increases the risk for about 100 times
• A weakened immune system, due to organ transplants, HIV/AIDS, or autoimmune diseases, raises the risk
• Having cancer, especially blood cancer