Stevens-Johnson Syndrome: Do You Have a Medical Malpractice Case?
What is Stevens-Johnson syndrome?
Stevens-Johnson syndrome (SJS) and the related condition toxic epidermal necrolysis (TEN) are serious and potentially life-threatening disorders that can affect your skin and the moist inner lining of certain organs.
SJS/TEN are rare skin reactions, but when they occur, they are considered medical emergencies and usually require hospitalization. These two conditions exist on a spectrum. While SJS is indeed a serious medical emergency, TEN is even more severe and life-threatening.
Flu-like symptoms – fatigue, fever, sore mouth and throat, cough, and burning eyes – are usually the first signs of SJS/TEN. Skin-related symptoms occur later. A painful reddish-purple rash spreads and turns into blisters. These blisters can appear on the lining of the mouth, nose, eyes, and genitals.
After blistering, the outer layer of skin dies, peels, and eventually heals. The extent of skin involvement distinguishes SJS from TEN:
- SJS is a skin peeling on 10% or less of the body.
- SJS & TEN overlap and are defined as skin peeling on 10-30% of the body.
- TEN is defined as skin peeling on more than 30% of the body.
SJS/TEN can occur at any age, but most frequently affects children and young adults (ages 20-40 years old). People with history of bone marrow transplants, systemic lupus erythematous (SLE), or chronic joint diseases are at increased risk of developing the condition.
What are the expected outcomes for someone with SJS/TEN?
In the short term, SJS/TEN can be debilitating and painful. Sores on the linings of the mouth, throat, genitals, and eyes can make normal bodily functions difficult. For example, it can be difficult for affected individuals to eat or close their mouths. Their eyes can become swollen shut. Sores in the lining of the digestive and respiratory tracts can cause diarrhea, pneumonia, and make it difficult to breathe.
Because skin loss is a primary characteristic of SJS and TEN, subsequent fluid loss, organ failure, and infection are significant concerns.
SJS and TEN are short-term conditions, but they can cause lasting, chronic effects. After recovery, a person may have to cope with difficult and expensive health outcomes for the rest of their lives:
- Infections like cellulitis (skin infection) and sepsis (potentially fatal bloodstream infection)
- Damage to the cornea, possibly resulting in loss of vision
- Respiratory disorders like acute respiratory failure and chronic obstructive pulmonary disease (COPD)
- Permanent skin damage (bumps, pigmentation, abnormal nail growth, itching, sweating, and dryness)
Unfortunately, some people who develop SJS/TEN do not survive the condition and related complications. The death rate associated with SJS is 5%. The death rate associated with TEN is up to 25% in adults and less than 10% in children.
What causes SJS/TEN?
Seventy-five percent of SJS/TEN cases are caused by medications, while 25% are caused by certain conditions, infections, or have an unknown cause (idiopathic SJS/TEN). Despite these statistics, the likelihood of developing SJS/TEN is typically due to a combination of genetic, environmental, and immunological factors. Certain groups of people are more likely to develop SJS/TEN:
- Weakened immune system
- Personal history or family history of SJS/TEN
- Belonging to an ethnic group that carries the HLA-B 1502 gene (e.g., Chinese, Southeast Asian, Indian)
Infections that have been known to precipitate SJS/TEN include:
- Herpes simplex (genital herpes or cold sores) or herpes zoster (shingles)
- Hepatitis A
Other conditions that could cause SJS/TEN include graft-versus-host disease (GVHD) and some vaccinations.
Antibiotics like penicillin are the most common medication triggers of SJS/TEN, but there are several other medications that can cause the disease:
- Anti-gout medications like allopurinol
- Anticonvulsants like phenytoin (Dilantin), carbamazepine (Tegretol), lamotrigine (Lamictal), valproic acid (Depakote) and phenobarbital
- Cough and cold medications
- Nevirapine, an HIV drug
- NSAIDs, especially those that end with -oxicam
What does treatment of SJS/TEN involve?
SJS/TEN treatment is intense and expensive. Almost always, a person who develops either condition will be hospitalized.
First, the doctor will discontinue all non-essential medications to try to determine the cause. The core of treatment will consist of wound care to prevent infections, eye care, and fluid replacement. Pain medications, anti-inflammatory drugs, and antibiotics may also be required.
Expensive medications and procedures may also be necessary:
- A medication called cyclosporine may help shorten the duration of blistering.
- Immune globulins can help block antibodies to prevent additional damage.
- A plasma exchange may help remove substances from the blood that may have caused SJS/TEN.
Beyond the burdensome length of hospitalization, the type of care the affected person requires also adds to the expense. Because of the nature of SJS/TEN, the patient will need a specialist doctor and placement in an intensive care unit (ICU) and/or burn unit. Skin regrowth can take 2-3 weeks, but full recovery may not be complete for months.
Do I have a medical malpractice case?
Families who have been impacted by Stevens-Johnson syndrome or toxic epidermal necrolysis unfortunately understand how dire circumstances can become. Without prompt, emergent treatment, people who develop SJS or TEN may not have the opportunity to recover completely or at all.
Furthermore, the short-term and long-term expenses of caring for someone who has experienced SJS or TEN can be prohibitive.