Genitourinary involvement and management in children with Stevens-Johnson syndrome and toxic epidermal necrolysis

J. P. Van Batavia*, D. I. Chu, C. J. Long, M. Jen, D. A. Canning, D. A. Weiss

*Corresponding author for this work

Research output: Contribution to journalArticle

5 Scopus citations

Abstract

Background: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are devastating hypersensitivity disorders that cause epidermal cell death and can affect all epidermal surfaces, including the urethra, vagina, labial and scrotal skin. Despite the well-described ocular and orofacial manifestations of SJS/TEN, there is a paucity of reports on the genitourinary (GU) symptoms and their management. Specifically, consulting services often ask the pediatric urology team if it is safe to place a urethral catheter, but there is no data in the literature to help guide management. The present study sought to review all pediatric cases of SJS/TEN in a tertiary care hospital to determine the incidence and optimal management of GU manifestations, including the use of urethral catheters. Methods: With IRB approval, cases of SJS and TEN that were managed as an inpatient between January 2008 and June 2015 were retrospectively reviewed in order to identify the extent of GU involvement/manifestations, the treatment provided, use of urethral catheterization and long-term follow-up or complications. Results: Thirty-one patients (15 female, 16 male; age range 2-18 years) presented with SJS or TEN over the study period. Etiologies for SJS/TEN included mycoplasma infection (48%) and medications (45%). Incidences of GU manifestations at presentation and their management are shown in Summary Table. Overall, 74% of patients had genital involvement of skin lesions. In 12 cases (39%), urology consultation was obtained. Twenty patients (61%) complained of dysuria and one child had gross hematuria in the setting of meatal lesion. Petroleum jelly was used in the majority of patients. A urethral catheter was placed in eight patients (25.8%, four female, four male) with a range of duration of 7-23 days. No patient developed hematuria or any other complications (i.e. strictures or urinary symptoms) after catheter removal. One boy required lysis of penile adhesions in the short-term. One of each gender developed penile and labial adhesions on long-term follow-up that self-resolved. Conclusions: GU involvement in SJS/TEN occurred in almost three-quarters of patients and was managed conservatively like other skin/mucosal manifestations. Long-term sequelae were rare and urethral catheterization appeared to be safe, without any short-term or long-term complications.Summary TableGenitourinary manifestations and management of Stevens-Johnson syndrome and toxic epidermal necrolysis in children.Summary TableVariables a OverallMalesFemales P-value** N 311615-Age at presentation, years0.62 Median (IQR)10.9 (7.4-14.1)10 (8-14)13 (6.7-14.5) Etiology of SJS 0.68 Mycoplasma15 (48.4)9 (56.3)6 (40.0) Lamotrigine4 (12.9)1 (6.3)3 (20.0) Sulfamethoxazole/Trimethoprim4 (12.9)1 (6.3)3 (20.0) Azithromycin3 (9.7)2 (12.5)1 (6.7) Amoxicillin2 (6.5)1 (6.3)1 (6.7) Valproic acid1 (3.2)1 (6.3)0 (0) Recurrent SJS1 (3.2)0 (0)1 (6.7) Unknown1 (3.2)1 (6.3)0 (0) Signs/Symptoms Dysuria20 (64.5)10 (62.5)10 (66.7)1.00 Hematuria1 (3.2)1 (6.3)0 (0)1.00 Urinary retention5 (16.1)3 (18.8)2 (13.3)1.00 Scrotal/labial lesions11 (35.5)5 (31.3)6 (40.0)0.72 Penile/vulvar lesions18 (58.0)7 (43.8)11 (73.3)0.15 Meatal lesions12 (38.7)9 (56.3)3 (20.0)0.07 Acute kidney injury1 (3.2)1 (6.3)0 (0)1.00 Management Urology consult12 (38.7)7 (43.8)5 (33.3)0.72 Foley urethral catheter8 (25.8)4 (25.0)4 (26.7)1.00 Topical petroleum jelly20 (64.5)9 (56.3)11 (73.3)0.46 Phenazopyridine3 (9.7)3 (18.8)0 (0)0.23 Topical lidocaine1 (3.2)1 (6.3)0 (0)1.00 Surgery1 (3.2)1 (6.3)0 (0)0.49 Long-term complications Penile/labial adhesions3 (9.7)1 (6.3)2 (13.3)1.00SJS = Stevens-Johnson syndrome; TEN = toxic epidermal necrolysis; IQR = interquartile range. ** P-values compared males vs. females with Wilcoxon rank-sum test for age and Fisher's exact test for all other variables.aValues shown are number (%) unless otherwise specified.

Original languageEnglish (US)
Pages (from-to)490.e1-490.e7
JournalJournal of Pediatric Urology
Volume13
Issue number5
DOIs
StateAccepted/In press - Sep 14 2016

Keywords

  • Genitourinary
  • Pediatrics
  • Stevens-Johnson syndrome
  • Toxic epidermal necrolysis
  • Urinary catheter

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Urology

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