Stevens-Johnson Syndrome (Symptoms, Diagnosis, Treatment, and Prevention)

Stevens-Johnson Syndrome (Symptoms, Diagnosis, Treatment, and Prevention)

Definition: Stevens-Johnson syndrome (SJS) is a rare but serious disorder that affects the skin, mucous membrane, genitals, and eyes. Together with toxic epidermal necrolysis (TEN) and Stevens-Johnson/toxic epidermal necrolysis (SJS/TEN), it forms a spectrum of disease, with SJS being less severe. Often, it begins with flu-like symptoms, followed by a painful red or purplish rash that spreads and blisters. Then the top layer of the affected skin dies, sheds and then heals. Complications include dehydration, sepsis, pneumonia, and multiple organ failure.

The most common cause is certain medications such as lamotrigine, carbamazepine, allopurinol, sulfonamide antibiotics, and nevirapine. Other causes can include infections such as Mycoplasma pneumonia and cytomegalovirus or the cause may remain unknown. Risk factors include HIV/AIDS and systemic lupus erythematosus.

SJS/TEN often is triggered by certain medications including allopurinol, anti-epileptics, pain relievers, cancer therapies, or antibiotics (sometimes up to 2 weeks after stopping the medication). SJS/TEN can also be triggered by infections such as pneumonia, herpes virus, and hepatitis A. In many cases the cause cannot be identified.

It is twice as common in males as females. Typical onset is under the age of 30. Skin usually regrows over two to three weeks; however, complete recovery can take months. Overall, the risk of death with SJS is 5 to 10%.

Stevens-Johnson syndrome is a medical emergency that requires treatment in a hospital, often in intensive care or a burns unit. Treatment aims to identify the underlying cause, control the symptoms and prevent complications.

Recovery after Stevens-Johnson syndrome can take weeks to months, depending on the severity of your condition. If it was caused by a medication, people will need to permanently avoid that drug and others closely related to it.

Stevens-Johnson syndrome (SJS) is named for Albert Mason Stevens and Frank Chambliss Johnson, American pediatricians who jointly published a description of the disorder in the American Journal of Diseases of Children in 1922.

Signs and Symptoms of Stevens-Johnson syndrome: Stevens-Johnson syndrome (SJS) usually begins with fever, sore throat, and fatigue, which is commonly misdiagnosed and therefore treated with antibiotics. Within about 1 to 3 days, a reddish or purplish rash forms and the skin begins to blister and peel (detach), leading to “raw” areas of skin that are painful.

Flu-like symptoms are also usually present during the initial stages, and may include:

  • feeling generally unwell
  • a high temperature (fever) of 38C (100.4F) or above
  • a headache
  • joint pain
  • a cough

A rash of round lesions about an inch across arises on the face, trunk, arms and legs, and soles of the feet, but usually not the scalp.

The mucous membranes (thin, moist tissues that line body cavities) of the mouth, throat, genitals, eyes, and/or digestive tract usually also are involved and may become affected before or after the skin. Early symptoms of mucous membrane involvement may include light sensitivity, itching or burning of the eyes, and pain when swallowing.

Symptoms may persist or worsen for up to two weeks before the skin and mucous membranes begin to start healing themselves. Skin that remained attached may gradually peel during this time, and the nails may shed in some cases.

Diagnosis and Treatment of Stevens-Johnson syndrome: Stevens-Johnson syndrome (SJS) should be diagnosed by a dermatologist (skin specialist). The diagnosis is based on the involvement of less than 10% of the skin. A positive Nikolsky’s sign is helpful in the diagnosis of SJS and TEN. A skin biopsy is helpful, but not required, to establish a diagnosis of SJS and TEN.

Tests and procedures used to diagnose Stevens-Johnson syndrome include:

  • Physical exam
  • Blood tests
  • Skin biopsy
  • Culture
  • Imaging

A person suspected of having Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) should be admitted to the hospital immediately because it can be life-threatening. When the diagnosis is confirmed, the extent of the disease should be determined quickly so that the most appropriate place for treatment can be decided. People with SJS/TEN may be best treated in an intensive care unit, burn unit or specialized dermatology unit.

Treatment for the relief of symptoms while in a hospital may include:

  • strong painkillers – to help ease the pain of any raw areas of skin
  • cool, moist compresses held against the skin – dead skin may be gently removed and a sterile dressing placed over the affected area
  • regularly applying a plain (unscented) moisturizer to the skin
  • replacement fluids – people may receive fluids and nutrition through a tube that’s passed through their nose and into their stomach (a nasogastric tube)
  • mouthwashes containing anesthetic or antiseptic – to temporarily numb their mouth and make swallowing easier
  • a short course of corticosteroid tablets (topical corticosteroids) to control skin inflammation (only on specialist advice)
  • antibiotics – if blood poisoning (sepsis) is suspected
  • eyedrops or eye ointment – for eye-related symptoms

Treatment aims to address symptoms and prevent complications (supportive care). People thought to have medication-induced SJS/TEN should discontinue the medication as soon as possible.

Preventions of Stevens-Johnson syndrome: Screening individuals for certain predisposing gene variants before initiating treatment with particular SJS-, TEN/SJS-, or TEN-inducing drugs is recommended or under study. If Stevens-Johnson syndrome has been caused by an adverse reaction to a medication, people will need to avoid taking this medication and other similar medications.

If people are of Chinese, Southeast Asian or Indian descent, genetic testing may be recommended before taking medications known to have an associated risk of causing Stevens-Johnson syndromes, such as carbamazepine and allopurinol.

Testing will help determine whether you carry the genes (HLA B1502 and HLA B1508) that have been associated with the syndrome when taking these medications.

It has also been suggested that all individuals found to express this HLA serotype avoid treatment with abacavir. Current trials are underway in Taiwan to define the cost-effectiveness of avoiding phenytoin in SJS, SJS/TEN, and TEN for individuals expressing the CYP2C9*3 allele of CYP2C9.

 

Information Source:

  1. nhs.uk
  2. rarediseases.info.nih.gov
  3. mayoclinic.org
  4. wikipedia