Is difficulty urinating a symptom of Stevens-Johnson Syndrome (SJS)?

From the Guidelines

Difficulty urinating can be a symptom of Stevens-Johnson Syndrome (SJS), particularly in the form of dysuria or urinary retention, due to the involvement of the urogenital tract. SJS is a rare, serious skin and mucous membrane disorder characterized primarily by widespread skin pain, a red or purple skin rash that spreads, and blistering of the skin and mucous membranes, particularly in the mouth, nose, eyes, and genitals. The main symptoms include fever, sore throat, fatigue, cough, burning eyes, and the characteristic skin rash that eventually leads to skin sloughing. According to the UK guidelines for the management of SJS/toxic epidermal necrolysis in adults 2016 1, involvement of the urogenital tract is common and can lead to serious morbidity, including strictures and stenosis of the urethra, phimosis in males, and vaginal synechiae in females, resulting in urinary and sexual dysfunction.

The guidelines recommend catheterizing all patients to prevent strictures forming in the urethra 1. Additionally, the study published in the British Journal of Dermatology in 2016 1 notes that urinary dysfunction, including dysuria or retention, is common during the acute phase of SJS/TEN, and that secondary infection by bacteria or candida is a frequent complication of urogenital involvement. Therefore, it is essential to consider difficulty urinating as a potential symptom of SJS, particularly in patients with urogenital tract involvement, and to provide prompt and appropriate management to prevent long-term complications.

Key considerations in the management of SJS include:

• Regular examination of the urogenital tract
• Application of white soft paraffin ointment to the urogenital skin and mucosae
• Use of Mepitel dressings to eroded areas in the vulva and vagina
• Consideration of potent topical corticosteroid ointment to the involved, noneroded, urogenital surfaces
• Catheterization to prevent strictures forming in the urethra 1.

From the Research

Symptoms of Stevens-Johnson Syndrome (SJS)

• SJS is a rare but serious disorder that can cause epidermal cell death and affect all epidermal surfaces, including the urethra, vagina, labial, and scrotal skin 2
• The syndrome can lead to various genitourinary (GU) symptoms, including:
  • Dysuria (painful urination) 2, 3
  • Urinary retention 3
  • Hematuria (blood in the urine) 2, 4
  • Obstructive uropathy 4
• These symptoms can be a result of the mucocutaneous hypersensitivity disorders characterized by sudden onset epidermal necrosis, which can lead to complications such as vulvovaginal adhesions, vaginal stenosis, or dryness 3

Management and Treatment of SJS

• The treatment of GU manifestations in SJS relies largely on expert opinion, and there is little data on the efficacy of suggested management 3
• Urethral catheterization may be necessary in some cases, and it appears to be safe without any short-term or long-term complications 2
• Early recognition and treatment of vulvovaginal involvement are crucial to preventing severe sequelae, and the use of vaginal dilators and steroid ointment may be recommended 3
• Symptomatic treatment, including skin care, anti-infection therapy, and high-dose glucocorticoid treatment, is often used to manage SJS 5

Complications and Long-term Sequelae

• SJS can lead to various long-term sequelae, including difficulty with vaginal exams, psychological distress, vaginal dryness, pain, and dyspareunia 3
• The impact of treatment in the acute setting on the development of chronic sequelae needs to be established, and the management of long-term sequelae is an area that requires further research 3