Title : A unique case of stevens-johnson syndrome/toxic epidermal necrolysis complicated by pelvic organ prolapse
Abstract:
Aim: The aim of this case is to provide novel insight into the management and outcomes of patients with Stevens-Johnson Syndrome and Topic Epidermal Necrolysis extending to gynecological mucosa.
Background: To date, there is limited knowledge of patients with Stevens-Johnson Syndrome (SJS) or Toxic Epidermal Necrolysis (TEN) extending to gynecologic mucosa, and there is limited documentation of patients with SJS/TEN in the setting of pelvic organ prolapse (POP). SJS is very rare, affecting between 1-2 people per million worldwide each year. Of these patients, the prevalence of acute vulvovaginal involvement is up to 70%, while 28% of patients even develop chronic vulvovaginal sequelae. This disease is life-threatening and complicates medical management, but its rarity has led to a paucity of treatment guidelines for patients with gynecologic sequelae.
Methods: This study is a case report with a review of the current literature on the reported cases of SJS/TEN including the vulva and vaginal mucosa. This case describes a history of history of erythematous rash and desquamation with increasing involvement including the vulva and vaginal mucosa as a manifestation of severe SJS/TEN in the setting of Stage 4 non-reducible POP. We searched PubMed for all English language articles from 2000 to 2023, with search terms including “Stevens-Johnson Syndrome of vulva,” “StevensJohnson Syndrome of vaginal mucosa,” “Stevens-Johnson Syndrome and pelvic organ prolapse,” “Toxic Epidermal Necrolysis of vulva,” “Toxic Epidermal Necrolysis of vaginal mucosa,” and “Toxic Epidermal Necrolysis and pelvic organ prolapse.”
Results: This patient’s case included a history of erythematous rash on her abdomen, waist, buttocks, and oral cavity; pain with urination; and burning of her vulva. Physical examination demonstrated SJS in the setting of stage 4 non-reducible POP, which was treated with clobetasol and estrogen cream with her daily dressing changes. Dressing wraps including covering around her prolapsed organs, and a foley catheter was placed due to the patient’s severe pain with urination. As the patient’s admission progressed, she declined drastically, with increasing creatinine, tachycardia to the 120s, and confusion/disorientation. The patient’s oxygen requirement was also increasing, and she was upgraded to the ICU on day 6 of admission. Although an uncomplicated intubation was completed, her oxygen saturation remained in the 50s, prompting investigation with a bronchoscope which revealed diffuse alveolar hemorrhages. Despite the team’s respiratory support efforts, she continued to experience repetitive desaturations. She ultimately developed ventricular tachycardia reaching 180 beats-per-minute. Due to the patient’s wishes, CPR/ACLS was not applied. Her family members withdrew care and were present at bedside during her passing.
Conclusion: This case provides an opportunity for novel insight into the management of patients with SJS/TEN in addition to gynecologic sequelae. It also reveals future points of investigation; it is essential to follow-up with patients who present with SJS/TEN with POP to analyze any further gynecologic sequelae of SJS/TEN following treatment of the prolapse. Additionally, it is imperative for prospective studies to assess long term impacts on quality of life and sexual function, as there is a paucity of knowledge on this subject as well.
Audience Take Away:
- Early recognition and diagnosis of SJS/TEN with gynecologic involvement is imperative in preventing internal progression as well as chronic sequelae resulting from adhesions and agglutination of the vulva and vagina.
- Using the known treatment for patients with vulvovaginal involvement of SJS/TEN which consists of dressing changes with steroid cream and estrogen cream in addition to the use of a foley catheter is likely to prevent internal progression of SJS/TEN.
- Prospective studies are needed to evaluate the long-term gynecologic sequelae of patients with SJS/TEN, including the long-term impacts on quality of life and sexual function.
