Quick Consult: Symptom: Facial Swelling: Emergency Medicine News
A 23-year-old woman with no history presented with facial swelling. She was taken by ambulance and on arrival stated that the swelling had started a few days earlier.
She noted that she had started antibiotics for a cyst 10 days earlier and then developed a sore throat and vaginal pain. She went to her doctor’s office three days before her emergency room visit and started Flagyl. The patient said the swelling had gotten worse and she developed a rash.
Her temperature was 37.7°C, heart rate was 114 bpm, and blood pressure was 98/56 mm Hg. She appeared unwell and had difficulty speaking. Her conjunctiva was inflamed and she said her throat hurt so much that she couldn’t eat or drink much. She also noticed swollen lips. What is the diagnosis?
Diagnosis and case discussion can be found on the next page.
Diagnosis: Stevens-Johnson syndrome
The patient’s rash with mucosal involvement was a significant concern for Stevens-Johnson syndrome (SJS). This patient had ocular, oral, and genital involvement. SJS is a significant mucocutaneous reaction most commonly caused by drugs. It causes extensive necrosis and detachment of the epidermis. (J Invest Dermatol. 2017;137:1004; https://bit.ly/3s1uAOs.)
It is often thought that SJS is on a continuum with toxic epidermal necrolysis (TEN). SJS is less severe because less than 10 percent of the body’s surface area is affected. Patients have TEN when more than 30 percent of their body is affected. SJS and TEN are not very common, but SJS is the more common variant. Mortality from SJS is about 10 percent and that from TEN can be as high as 50 percent.
The most common pathogens are drugs. The reaction usually occurs after four days to four weeks of continuous medication use. Patients often present with flu-like symptoms such as body aches, fever, cough, malaise, myalgia and blurred vision. (Up to date. Aug 10, 2021; http://bit.ly/2MpUy9d.) The patient may begin to develop a red or purple rash that develops into blisters. The skin becomes painful. Mucous membranes are usually affected in 90 percent of cases, and patients may have signs of conjunctivitis, difficulty swallowing, and genital pain. (Genetics Home Reference. July 2015; https://bit.ly/3pUYBwP.)
The severity and prognosis depend on the amount of skin affected. The SCORTEN scoring system can be used to predict patient prognosis. (J Invest Dermatol. 2000;115:149; https://bit.ly/3oPm5UB.)
The patient’s laboratory findings often include anemia and leukopenia. (J.Am. acad. dermatol. 1990;23[6 Pt 1]:1039.) Neutropenia often correlates with a poor prognosis. Other abnormal laboratory findings may include elevated blood urea nitrogen, elevated glucose, electrolyte abnormalities, and hypoalbuminemia. (J Invest Dermatol. 2000;115:149; https://bit.ly/3oPm5UB.)
These abnormalities can be found due to the fluid losses patients experience as a result of their skin lesions and hypovolemia. The acute phase of SJS and TEN can last eight to twelve days. These patients are at risk for several complications, including sepsis, pneumonia, and gastrointestinal disorders. Close monitoring is extremely important in this acute phase.
Patients with SJS and TEN require hospitalization. The severity of the disease must be determined in order to determine the best place to admit the patient. The SCORTEN score can be used to help with this decision. A SCORTEN score of 0-1, where the disease is not progressing rapidly, may not require specific treatment. Patients with body surface area involvement of 30 percent or more or a SCORTEN score of 2 or greater should be admitted to an intensive care unit, e.g. B. a burns intensive care unit. (J Burn Care Rehab. 2002;23:87.)
Management of these patients requires rapid discontinuation of the offending drugs and supportive measures that include wound care, IV fluids, nutrition, pain control, and prevention of infection when possible. The use of IVIG and steroids remains controversial.
This patient was quickly assessed upon arrival and admitted to our burns intensive care unit for IVF and pain medication.
Dr Kaplanis an assistant professor of emergency medicine at the University of Colorado School of Medicine at Aurora. Follow her on Twitter@bonniekaplan20, and read her past columnshttp://bit.ly/EMN-QuickConsult.