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

Summary 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 Table . Genitourinary manifestations and management of Stevens–Johnson syndrome and toxic epidermal necrolysis in children. Variables a Overall Males Females P -value** N 31 16 15 – Age at presentation, years 0.62 Median (IQR) 10.9 (7.4–14.1) 10 (8–14) 13 (6.7–14.5) Etiology of SJS 0.68 Mycoplasma 15 (48.4) 9 (56.3) 6 (40.0) Lamotrigine 4 (12.9) 1 (6.3) 3 (20.0) Sulfamethoxazole/Trimethoprim 4 (12.9) 1 (6.3) 3 (20.0) Azithromycin 3 (9.7) 2 (12.5) 1 (6.7) Amoxicillin 2 (6.5) 1 (6.3) 1 (6.7) Valproic acid 1 (3.2) 1 (6.3) 0 (0) Recurrent SJS 1 (3.2) 0 (0) 1 (6.7) Unknown 1 (3.2) 1 (6.3) 0 (0) Signs/Symptoms Dysuria 20 (64.5) 10 (62.5) 10 (66.7) 1.00 Hematuria 1 (3.2) 1 (6.3) 0 (0) 1.00 Urinary retention 5 (16.1) 3 (18.8) 2 (13.3) 1.00 Scrotal/labial lesions 11 (35.5) 5 (31.3) 6 (40.0) 0.72 Penile/vulvar lesions 18 (58.0) 7 (43.8) 11 (73.3) 0.15 Meatal lesions 12 (38.7) 9 (56.3) 3 (20.0) 0.07 Acute kidney injury 1 (3.2) 1 (6.3) 0 (0) 1.00 Management Urology consult 12 (38.7) 7 (43.8) 5 (33.3) 0.72 Foley urethral catheter 8 (25.8) 4 (25.0) 4 (26.7) 1.00 Topical petroleum jelly 20 (64.5) 9 (56.3) 11 (73.3) 0.46 Phenazopyridine 3 (9.7) 3 (18.8) 0 (0) 0.23 Topical lidocaine 1 (3.2) 1 (6.3) 0 (0) 1.00 Surgery 1 (3.2) 1 (6.3) 0 (0) 0.49 Long-term complications Penile/labial adhesions 3 (9.7) 1 (6.3) 2 (13.3) 1.00 SJS = 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. a Values shown are number (%) unless otherwise specified.