When It’s Not a Burn

Two medical workers perform their duties on a patient with a burn injury in a hospital bed. — Photo provided by the author.

Scenario

You are called to check the well-being of a sixty-one-year-old female who lives by herself and whose family has not been able to contact her. You enter the apartment with police and fire personnel and find the woman unconscious on her sofa.

She is breathing, has a pulse and stable vital signs but her face and arms are covered by what look like first and second degree burns, so you treat her appropriately.

On inspection of the apartment, however, no one can smell any smoke, there is nothing left on the stove, and there is no charring or other evidence of any fire, so you are curious as to what the mechanism of the burns was.

Stevens-Johnson Syndrome

U.S. Air Force Airman 1st Class Mindy Hurley, 81st Aerospace Medicine Squadron mental health technician, poses with a simulated injury during moulage training inside Wolfe Hall at Keesler Air Force Base, Mississippi, Jan. 29, 2020. Moulage, also known as combat injury simulation, is used in base exercises to simulate the outcome of various situations such as chemical burns, piercing wounds and missing limbs using different tools such as latex, stage blood, sponges and makeup products. — Photo/Airman 1st Class Kimberly Touchet

In this scenario, you were actually looking at a medical condition, there had been no thermal, chemical, or electrical injury to the patient.

This woman was presenting with a medical condition that can cause painful blisters and lesions on the skin and mucous membranes, can cause severe eye problems, and can present similarly to a first or second degree burn. It is known as Stevens-Johnson Syndrome (SJS) and the most common cause is an adverse drug reaction.

Stevens-Johnson Syndrome (SJS) and similarly, toxic epidermal necrolysis (TEN) are two forms of the same life-threatening skin disorder and are serious conditions that cause skin to develop rashes, blisters, and then peel.

Some literature identifies SJS and TEN to be different diseases, while others consider them to be the same disease but at different levels of severity. SJS is less intense than TEN, for example, skin peeling might affect less than 10% of the entire body in SJS, while skin peeling affects more than 30% of the body in TEN.¹

However, both conditions can be life-threatening, and you may also see them referred to as Lyell’s Syndrome, named after the man who first described the condition.

Many cases of SJS happen in children and adults younger than 30 years old, but also occur in others, especially the elderly. More cases of SJS occur in females than males.

Infections, like pneumonia, are the most likely cause of SJS in children, whereas medications are the most likely cause of SJS/TEN in adults. SJS is more common in children and younger adults but can develop at any age.

Signs and Symptoms

A common presentation is for a person to complain of flu-like symptoms, such as fever, sore throat, cough or joint pain, then a few days later, a skin rash appears somewhere on the body that feels like a sunburn.

Part of the difficulty in assessing SJS is that many of the signs and symptoms are vague or can mimic other common conditions.

Other symptoms of SJS, which appear as the condition progresses, may include:

Blisters or swelling on the skin, mouth, eyes, nose or genitals
Desquamation after blisters
Allodynia

SJS can also severely affect the eyes. Typical eye problems associated with SJS can include conjunctivitis ulceration of the eyelids, iritis, or corneal blisters or perforation.

Figure 1 lists signs and symptoms of Stevens-Johnson Syndrome, but it should be noted that each patient will respond differently. It may also be important to remember that SJS is not contagious.

Fever	Body aches
Red rash or blotches on the skin	Cough
Peeling skin	Drooling (because closing the mouth is painful)
Eyes sealed shut due to swelling	Painful urination

Figure 1

Causes

Almost any drug can result in SJS, but medicines such as antibiotics, cold medicines, anticonvulsants, and anti-inflammatory treatments have been the most frequent causes, although a combination of factors is more likely involved in developing these disorders. Genetic influence is thought to play a role.

One of these genetic factors includes specific human leukocyte antigens (HLAs) that may increase one’s risk of developing SJS or TEN.² Environmental factors might also cause the gene to be triggered. For example, the condition seems to be more commonly present in Spring months.

Medications that seem most likely to cause Stevens-Johnson syndrome include:

Antibacterial sulfa drugs.
Anti-epileptic drugs.
Non-steroidal anti-inflammatory drugs (NSAIDs)
Antibiotics.

Further, people are at greater risk of SJS if they have one of the following conditions:

Bone marrow transplant.
Systemic lupus erythematosus.
Other chronic diseases of joints and connective tissue.
Cancer.
Weakened immune system.
Family history of SJS.

SJS may redevelop if a person is exposed to the same medication known to have triggered the condition the first time. In such cases, the second episode is usually more severe than the first episode.

The mortality rate of SJS and TEN ranges from approximately 10% to 34%³ due mostly to sepsis, acute respiratory distress syndrome and multiple organ failure.

Management

Two healthcare workers perform patient care on a patient in a hospital bed. — Photo provided by the author.

A diagnosis of SJS must be done with a skin biopsy, imaging and blood tests, but if you were to respond to a scenario, and based on a review of the medical history and physical exam you were to suspect SJS, the immediate treatment should include,

Immediate airway management
Transport any medications that are visible, open, or appear to be new to the ED.
Placement of an IV in an unaffected area.
Using non-adhesive dressings on the affected skin.
Pain management
Obtaining medical consultation to determine most appropriate receiving facility.

Remember to prioritize the airway. Not all burn patients present with airways that require intubation, especially in the prehospital environment. An alert and oriented patient with no respiratory distress and no visible airway injury is unlikely to need emergent intubation.

A patient with suspected SJS should be approached similarly. If there are concerns about airway edema, blisters or sloughing, a patient should be intubated before the airway becomes compromised. The decision to intubate a possible SJS patient at the scene will be dependent upon EMS providers’ assessment, protocols, and medical direction.

If the patient is in arrest or unconscious without a gag reflex, EMS providers should immediately provide bag-valve-mask ventilation and consider intubation. If the patient is unconscious but still has a gag reflex, or remains conscious with airway compromise, RSI could be considered if available. If RSI is not an option, then 100% oxygen via face mask should be administered.

Pearls

Stevens-Johnson Syndrome can easily be mistaken for a burn injury because it presents with similar erythema, blisters, peeling skin and swelling as seen in first or second degree burns.
Management of patients with Stevens-Johnson Syndrome is usually provided in intensive care units or burn centers. Many patients are treated symptomatically, but management of SJS is often similar to the management of burns.
EMS providers should recognize the presence of severe fluid loss and can treat patients with Stevens-Johnson Syndrome as they would patients with thermal burns.
There is no way to prevent SJS. Since, in most cases, it is triggered by a medication, there is no way to know, prior to taking the medication, that an adverse reaction may occur.
Proper PPE should be worn, but SJS cannot be spread from person to person.