Genitourinary involvement and management in children with Stevens-Johnson syndrome and toxic epidermal necrolysis.
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.
Division of Urology, The Children's Hospital of Philadelphia, Philadelphia, PA, USA. Electronic address: vanbatavij@email.chop.edu.
J P
JP
Van Batavia
Division of Urology, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.
D I
DI
Chu
Division of Urology, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.
C J
CJ
Long
Section of Dermatology, The Children's Hospital of Philadelphia, Philadelphia, PA, USA; Department of Pediatrics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA; Department of Dermatology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA.
M
M
Jen
Division of Urology, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.
D A
DA
Canning
Division of Urology, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.
D A
DA
Weiss
D000293:Adolescent; D000367:Age Factors; D002648:Child; D015331:Cohort Studies; D015897:Comorbidity; D019468:Disease Management; D005260:Female; D052776:Female Urogenital Diseases / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology*; D005500:Follow-Up Studies; D006801:Humans; D015994:Incidence; D008297:Male; D052801:Male Urogenital Diseases / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology*; D010577:Petrolatum / Q000494:pharmacology*; D012189:Retrospective Studies; D018570:Risk Assessment; D012720:Severity of Illness Index; D012737:Sex Factors; D018709:Statistics, Nonparametric; D013262:Stevens-Johnson Syndrome / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology*; D062606:Tertiary Care Centers; D016896:Treatment Outcome
D010577:Petrolatum
Genitourinary; Pediatrics; Stevens–Johnson syndrome; Toxic epidermal necrolysis; Urinary catheter