An epidemiologic overview of a tertiary referral practice for male paediatric lichen sclerosus

Abstract

Within the paediatric population, changing patterns of circumcisions have confounded the epidemiology and presentation of lichen sclerosus (LS). We sought to evaluate the incidence, demographics, and clinical features of patients presenting to a single Albertan paediatric urologist with LS. This retrospective descriptive analysis evaluated all paediatric patients referred for phimosis to a single paediatric urologist in Edmonton, Alberta. Chief complaints/symptoms, date of birth, and date of circumcision were identified. The primary outcome of interest was the proportion of circumcisions with pathologically confirmed LS. From July 2006 to March 2016, 4,163 patients were seen for phimosis of the approximate 12,000 new referrals. Hundred phimosis patients had clinically suspected LS. Of those adequately reported, 81 (81/83) were microscopically confirmed to be LS with a mean age of 9.6 years and median age of 8.9 years (range 4.1 to 16.1 years). This cohort represented 2.0% of phimosis referrals and approximately 0.7% of all referrals to our paediatric urologist. When compared to physiologic phimosis, these patients had higher rates of dysuria (n=28, 34.6% versus n=1, 1.0%, P<0.0001) and urinary retention (n=18, 22.2% versus n=1, 1.0%, P<0.0001) as presenting complaints. LS of the paediatric male genitalia is an uncommon, albeit clinically significant disease entity. The clinical diagnosis for the trained practitioner is very accurate.

Comparison of lichen sclerosus in boys and girls: A systematic literature review of epidemiology, symptoms, genetic background, risk factors, treatment, and prognosis

“…The referral diagnoses for boys with LS are mostly clinical features such as phimosis (52%), balanitis (13%), or buried penis (10%), regardless of whether or not the LS has been recognized 14 31 the largest cohort studies reporting 10–50% 15,18,32,33 . Boys with an acquired phimosis may have a greater risk of having LS than boys with congenital phimosis 17,31,34 .…”

“…The perianal region of boys is rarely affected. Therefore, secondary constipation is uncommon 6,11,32,37,38 . Extragenital LS is present in 0.4–6% of boys with LS 6,11 …”

“…Hyperkeratosis (82.9%), hyalinization (100%), basal cell degeneration (56.1%), lymphocytic infiltration (100%), fibrin deposition (7.3%) 32 …”

“…Hypopigmentation, hyperpigmentation, erythema, fissures, atrophy, keratotic papules 19,22 Telangiectasias/purpura, angiokeratomas (rare) [23][24][25] Labial fusion 27,28 Hypopigmentation distal portion of the prepuce 35 Phimosis, scarring, secondary buried penis, balanitis Erythema, telangiectasias/purpura 11,35,36 Histopathology Hyperkeratosis (96%), basal vacuolization of keratinocytes (88%), lymphocytic exocytosis (91%), dermal sclerosis (99%) and epidermal atrophy (50%) 43 Hyperkeratosis (82.9%), hyalinization (100%), basal cell degeneration (56.1%), lymphocytic infiltration (100%), fibrin deposition (7.3%) 32 In 67% dermoepidermal clefts 44…”

“…14 The incidence of LS in non-circumcised boys with phimosis ranges from 2 to 95%, 31 the largest cohort studies reporting 10-50%. 15,18,32,33 Boys with an acquired phimosis may have a greater risk of having LS than boys with congenital phimosis. 17,31,34 Frequent symptoms subsequent to phimosis include urine retention, ballooning of the foreskin, dysuria, other urinary tract symptoms, and erectile pain.…”