Definition and Overview

Stevens-Johnson syndrome (SJS) is a rare but potentially life-threatening skin reaction. It is characterised by shedding or sloughing of skin and mucosal membrane, which makes it extremely difficult and painful for a person to swallow, pee or open their eyes fully. Due to the severity of its symptoms, it is important for patients to receive treatment promptly in an intensive care or burn unit.

SJS is commonly caused by an infection or severe allergic reaction to certain medications including chemotherapeutic agents and bipolar, anti-seizure, and antiretroviral medications. However, there are cases in which the exact cause is not identified. Treatment of the condition focuses on addressing the underlying cause and providing supportive care, including fluid replacement and wound and eye care.

The prognosis depends on the severity of symptoms and whether secondary infection occurs. When more than 30% of the body’s surface area is affected, the mortality rate is up to 35%.

Causes of Condition

Stevens-Johnson syndrome, which was first described by paediatricians Frank Chambliss Johnson and Albert Mason Stevens in 1922, can be triggered by certain infections and medications. Its symptoms may begin to show as soon as the offending drug has been ingested or up to two weeks after the drug has been discontinued.

Drugs and infectious diseases that can trigger the syndrome include:

  • Anti- gout medications

  • Antibiotics, including penicillin

  • Anticonvulsants and antipsychotics

  • Pain relievers, including naproxen sodium, Tylenol, and ibuprofen

  • Pneumonia

  • Herpes virus

  • Human immunodeficiency virus (HIV)

  • Hepatitis A

Some people are more prone to developing the condition than the general population. These are those who have the following risk factors:

  • A compromised immune system, which could be due to autoimmune diseases, surgical procedures such as an organ transplant, or those taking immunosuppressant drugs

  • A personal or family history of SJS

  • The HLA-B* 1502 gene - When combined with drugs for the treatment of gout or mental illness, this gene can increase the risk of SJS

Key Symptoms

The first symptoms of Steven-Johnson syndrome are fever, bloodshot eyes, sore throat, and fatigue. Because it shares the same symptoms with certain types of infections, it is not uncommon for patients to be misdiagnosed and initially treated with antibiotics. After around three to five days, other more serious symptoms, including widespread burning skin pain and blisters on the lips, mouth, tongue, and anal and genital regions begin to develop. A rash of round lesions also appears on the extremities and face.

Unless promptly diagnosed and treated, the condition can cause serious complications that produce more troubling symptoms. These include:

  • Skin infection

  • Sepsis – A medical emergency commonly caused by bacterial infection in the blood. Its symptoms include dizziness, confusion, slurred speech, severe muscle pain, and difficulty breathing.

  • Extensive tissue damage in the eye that can lead to significant visual impairment

  • Acute respiratory failure

  • Lasting skin problems, which may cause the hair to fall out and nails to grow abnormally

Who to See and Types of Treatments Available

People showing signs of Stevens-Johnson syndrome are often admitted to the intensive care or burn unit to adequately manage their symptoms and prevent additional serious complications from developing.

The condition is diagnosed with a number of medical tests and procedures, including complete blood count (CBC), physical examination, skin and blood culture, and skin biopsy.

The first and most important step is identifying the underlying cause of the syndrome. If it is caused by a certain drug, the patient is advised to stop taking it immediately. In the majority of cases, this causes the symptoms to subside.

Treatment of SJS typically focuses on providing supportive care that aims to provide pain relief and prevent symptoms from worsening as well as serious complications from developing.

  • Fluid replacement and electrolyte correction - SJS sufferers commonly lose an enormous amount of bodily fluid due to massive skin loss and because their blisters make it difficult for them to take in any food or liquid by mouth. The lost fluid can be replaced through an intravenous line or the use of a nasogastric tube.

  • Wound care - This aims to prevent infection and damage to underlying structures including the nerves, tendon, arteries, muscles, and bones. It involves the gentle removal of any dead skin and the use of medicated dressing over the affected areas.

  • Eye care - The patient’s medical team usually includes an ophthalmologist or an eye specialist who focuses on managing and treating eye problems that may arise due to the condition.

  • Medications - Pain medications are prescribed to reduce discomfort, antibiotics to treat infection, and steroids to reduce inflammation.

Stevens-Johnson syndrome has a mortality rate of 5%. The risk of death is higher in patients who develop acute organ failure and restrictive lung disease.

References:

  • De Rojas MV, Dart JK, Saw VP. The natural history of Stevens Johnson syndrome: patterns of chronic ocular disease and the role of systemic immunosuppressive therapy. Br J Ophthalmol. 2007 Aug. 91(8):1048-53.

  • Wetter DA, et al. Clinical, etiologic and histopathologic features of Stevens-Johnson syndrome during an 8-year period at Mayo Clinic. Mayo Clinic Proceedings. 2010;85:131.

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