A 23-year-old woman with no medical history presented with facial swelling. She was brought in by ambulance and said on arrival that the swelling had started a few days earlier.
She noted that she started taking an antibiotic for a cyst 10 days prior and then developed pain in her throat and vagina. She went to her doctor’s office three days before her ER visit and started taking Flagyl. The patient said the swelling got worse and she developed a rash.
She had a temperature of 37.7°C, a heart rate of 114 bpm, and a blood pressure of 98/56 mm Hg. She seemed uncomfortable and had difficulty speaking. Her conjunctiva was infected and she said her throat hurt so badly that she couldn’t eat or drink much. She also noted swollen lips. What is the diagnosis?
Find the diagnosis and case discussion 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 medications. It causes extensive necrosis and detachment of the epidermis. (J Invest Dermatol. 2017;137:1004; https://bit.ly/3s1uAOs.)
SJS is often thought to be part of a continuum with toxic epidermal necrolysis (TEN). SJS is less severe, with less than 10% of body surface area involved. Patients have TEN when more than 30 percent of their body is involved. SJS and TEN are not extremely common, but SJS is the most common variant. The mortality of SJS is about 10% and that of TEN can reach 50%.
The most common causative agents are drugs. The reaction tends to occur between four days and up to four weeks of continued medication use. Patients often experience flu-like symptoms such as body aches, fever, cough, malaise, myalgia, and blurred vision. (Up to date. August 10, 2021; http://bit.ly/2MpUy9d.) The patient may begin to develop a red or purple rash that will progress to blistering. The skin becomes painful. The mucous membranes are usually involved in 90% of cases, and patients may present with 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 involved. The SCORTEN scoring system can be used to help 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 is often correlated with a poor prognosis. Other abnormal lab results could include elevated blood urea nitrogen, high blood sugar, electrolyte abnormalities, and hypoalbuminemia. (J Invest Dermatol. 2000;115:149; https://bit.ly/3oPm5UB.)
These abnormalities can be found due to fluid losses that patients have as a result of their skin lesions and hypovolemia. The acute phase of SJS and TEN can last eight to 12 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 should be admitted to hospital. The severity of the disease must be determined to identify 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 specialist care. Patients with 30% or greater body surface area involvement or a SCORTEN score of 2 or greater should be admitted to an intensive care unit such as a burns intensive care unit. (J Burn Care Rehabilitation. 2002;23:87.)
Management of these patients requires prompt withdrawal of the offending drug and supportive care, which includes wound care, intravenous fluids, nutrition, pain control, and infection prevention when possible. The use of IVIG and steroids remains controversial.
This patient was promptly assessed upon arrival and admitted to our burn 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 its past columns athttp://bit.ly/EMN-QuickConsult.