Lichen Sclerosus: A Common but Often Overlooked Condition in Pediatric Patients

Introduction

Lichen sclerosus (LS) is a chronic inflammatory dermatosis that primarily affects the anogenital region, leading to epidermal atrophy, architectural distortion, and scarring. Although its exact pathogenesis remains unclear, contributing factors may include genetic predisposition, autoimmune processes, hormonal influences, infections, and mechanical trauma such as bicycle riding or frictional irritation.^1-3

Lichen sclerosus affects individuals of all ages and sexes but shows a strong female predominance, with reported female-to-male ratios ranging from 1:1 to 10:1. In girls, LS displays a bimodal age distribution, peaking in the prepubertal and postmenopausal periods. Among children, LS is most commonly diagnosed in girls aged 4 to 6 years.^1-3

In pediatric patients, especially girls, LS often presents with nonspecific urogenital symptoms such as dysuria, pruritus, vulvar pain, urinary incontinence, and recurrent urinary tract infections (UTIs). These symptoms are frequently misattributed to common pediatric urological conditions, leading to delays in diagnosis and unnecessary further invasive investigations, especially for recurrent UTIs. In addition, while symptoms may subside spontaneously after puberty, untreated LS may result in complications such as labial fusion, stenosis, or malignant transformation. ⁴

Timely recognition and treatment of LS is critical for symptom control, avoiding unnecessary invasive testing and preventing long-term sequelae. This study aimed to characterize the clinical features, diagnostic delays, and treatment outcomes of LS in children presenting with urinary complaints. Enhancing clinical awareness of LS in pediatric patients aims to minimize unnecessary invasive procedures.

Materials and Methods

Results

Demographic Data of Lichen Sclerosus Patients

Between January 2018 and December 2022, a total of 1018 pediatric patients presenting with urinary symptoms were evaluated for UTIs and voiding dysfunction at our center. Of these, 689 were female. Among the girls, 84 (12.2%) were diagnosed with LS. Including 4 boys diagnosed with LS due to phimosis, the total number of confirmed LS cases was 88.

The mean age at diagnosis was 6.06 ± 2.40 years. The median diagnostic delay (the time interval between the onset of symptoms and the establishment of LS diagnosis) was 8 months (mean = 8.42 ± 18.38 months). Contributing factors were identified in 78.4% of patients and included recurrent UTIs, mechanical trauma (eg, bicycle riding), comorbid autoimmune diseases, or a family history of LS or other autoimmune conditions (Table 1). Among the 9 patients with comorbid autoimmune disorders, autoimmune thyroiditis (n = 3), lichen planus (n = 2), and others (morphea, celiac disease, alopecia areata, psoriasis; n = 4) were reported. A positive family history of LS was noted in 3 patients; additional autoimmune conditions in relatives included autoimmune thyroiditis (n = 3), lichen planus (n = 2), psoriasis (n = 1), and alopecia areata (n = 1).

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Table 1.

Demographic Data of LS Patients.

Demographic data		Frequency (%)
Age, mean ± SD, years	6.06 ± 2.40
Delay of diagnosis a , mean ± SD, months	8.42 ± 18.38
Concomitant autoimmune disease (n)	9	10.2
Relationship b (n)	3	3.4
Family history of LS (n)	3	3.4
Family history of autoimmune disease (n)	7	7.9
History of cycling (n)	24	27.3
History of recurrent UTI (n)	68	78.4

a

Delay diagnosis: the time interval (in months) between the onset of symptoms reported by caregivers and the establishment of the LS diagnosis.

b

Relationship (n): refers to the number of patients with a positive family history of LS in first-degree relatives.

Clinical Findings

Dysuria was the most frequently reported symptom, initially present in 79.5% of patients and eventually in 100% during the disease course. Other urinary complaints included increased frequency, hematuria, and incontinence. Genital symptoms included genital itching (78.4%), whiteness, and occasional bleeding.

At the time of diagnosis, pyuria was observed in 87.5% of patients; however, only 45.5% had culture-proven UTIs. Escherichia coli was the most commonly isolated pathogen (Table 2). Approximately 25% of patients experienced recurrent UTIs during follow-up. Dysfunctional voiding was identified in 7 patients, commonly accompanied by constipation and daytime incontinence. All 7 had Dysfunctional Voiding Symptom Scores (DVSSs) >9, indicating clinically significant dysfunction.

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Table 2.

Clinical Findings of LS Patients.

	Frequency (n)	Frequency (%)
Presenting symptoms
Dermatological
Genital itching	69	78.4
Whiteness	12	13.6
Pain	11	12.5
Bleeding	8	9.1
Urinary system
Dysuria	70	79.5
Pollakiuria a	33	37.5
Hematuria	21	23.9
Incontinence	16	18.2
Urinary tract infection	40	45.5
Pyuria	77	87.5
Physical examination
Atrophy	83	94.3
Telangiectasia/erythema	83	94.3
Hypopigmentation	20	22.7
Scarring	1	1.3
Ecchymosis	1	1.3
Excoriation	1	1.3
LS therapy (topical)
Pimecrolimus	88	100
Tacrolimus	2	2.3
Steroid	2	2.3

a

Pollakiuria is defined as urination ≥8 times per day in children.

Clinical Course and Treatment Outcomes

Sixty patients who received treatment for at least 3 months were evaluated for treatment response. The mean follow-up duration was 16.83 ± 23.1 months. All patients initially received topical pimecrolimus. In cases of inadequate response (n = 4), 2 were switched to topical tacrolimus and 2 to topical corticosteroids.

Recurrence of dermatologic symptoms occurred in 4 patients (6.7%). Persistent urinary complaints were noted in 7 patients (11.7%), all of whom had documented dysfunctional voiding. Despite continued symptoms, DVSS scores significantly improved over time.

Overall, 66% of patients showed marked improvement in dermatologic findings, while 88% reported complete or substantial improvement in urinary symptoms, particularly within the first 3 months of treatment (Table 3).

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Table 3.

Progression of Followed LS Patients.

	Frequency (n)	Frequency (%)
During the follow-up
Frequent UTI	23	26.1
Constipation	7	8
Dysfunctional urination	7	8
After LS therapy (n = 60)
Dermatological findings
Improvement	40	66.6
Relapse	4	6.7
Urinary symptoms
Improvement	53	88.3
Persistence	7	11.7

Discussion

This study highlights that LS is an underrecognized yet relatively common cause of urinary symptoms, particularly dysuria, in pediatric patients. Importantly, the majority of patients responded well to early diagnosis and topical treatment, avoiding unnecessary invasive procedures.

Although the general population prevalence of LS ranges from 1 in 300 to 1 in 1000,^6,7 true rates may be underestimated due to misdiagnosis and low clinical awareness, especially in children. ⁶ In prepubertal girls, estimates suggest a prevalence of 1 in 900.^1-3 In our cohort, LS was diagnosed in 84 out of 689 girls (12.2%) presenting with urinary symptoms, suggesting a higher-than-expected frequency when appropriate screening and dermatological consultation are implemented.

The median diagnostic delay in our study was 8 months, which is shorter than reported in prior studies.^8,9 This may reflect increased awareness among pediatric nephrologists and urologists over time, as previously noted by others.^10,11 Enhancing awareness of LS across all pediatric specialties—including nephrology, urology, and general pediatrics—will be key to improving diagnostic accuracy and ensuring timely intervention.

Lichen sclerosus shows a clear female preponderance, with reported female-to-male ratios ranging from 1:1 to 10:1.^1-4,12,13 In our cohort, only 4 of 88 patients were male, all referred due to phimosis and diagnosed based on dermatological examination revealing atrophic white plaques at the external meatus (Figure 1). While phimosis is a typical presentation in boys, clinicians should maintain suspicion for LS in males with genital discomfort, itching, or dysuria, especially in the presence of erosive or ulcerative lesions. ¹⁴

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Figure 1.

Atrophic white plaques in the external meatus of a male pediatric patient with a history of surgery for hypospadias and lichen scleroatrophic lesions.

Consistent with previous studies, our findings support an association between LS and autoimmune diseases.^4,15 Both personal and family histories of autoimmune conditions were common, supporting the proposed autoimmune pathogenesis. Mechanical trauma—particularly from activities such as cycling—was also identified as a potential contributing factor.^10,16-18

Urinary symptoms were prominent, with dysuria present in all patients during the disease course. While adults more often report genital itching and pain, dysuria appears to be a dominant feature in pediatric LS. ⁴ Characteristic dermatologic findings included vulvar atrophy, erythema, telangiectasia, and hypopigmentation (Figure 2A-D), consistent with the literature.^8,10,19

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Figure 2.

(A) Bilateral erythematous telangiectatic and atrophic plaques in the genital area of a female pediatric patient with lichen scleroatrophic lesions. (B) White plaques with yellowish cystic papules on top, showing bilateral telangiectatic and atrophic lesions in the genital area of a female pediatric patient with lichen scleroatrophic lesions. (C) Bilateral telangiectatic and atrophic plaques in the genital area of a female pediatric patient with lichen scleroatrophic lesions. (D) Bilateral telangiectatic and atrophic white plaques in the genital area of a female pediatric patient with lichen scleroatrophic lesions and erosive areas at the posterior commissure of the labia.

The association between LS and recurrent UTIs has been previously documented. ²⁰ In our study, 87.5% of patients had pyuria, although only 45.5% had culture-confirmed infections, suggesting that LS-related inflammation may mimic UTI symptoms. Thus, LS should be considered in girls with persistent dysuria and sterile pyuria.

Incontinence and voiding dysfunction were also prevalent. Local inflammation and pain may exacerbate voiding symptoms.^21,22 Lichen sclerosus may not only mimic urinary conditions but also perpetuate them via irritation and treatment challenges.^22-24 In our patients with documented dysfunctional voiding, concurrent management—including urotherapy—resulted in improvement of both urinary and dermatologic outcomes, as evidenced by declining DVSS scores.

Topical corticosteroids are the standard treatment for LS. However, calcineurin inhibitors such as pimecrolimus and tacrolimus offer effective alternatives with favorable safety profiles and lower risk of atrophy and secondary infections, especially in children. ⁴ Our findings confirm the efficacy of pimecrolimus in the majority of cases, with early symptom relief often occurring within the first 3 months. A small proportion of patients required escalation to other topical agents due to inadequate response or recurrence.

Limitations of the study include its retrospective design, relatively small sample size—especially for dysfunctional voiding—and lack of a control group. Additionally, the follow-up duration was variable. Nonetheless, this is one of the few studies in a pediatric nephrology setting to systematically assess LS in children presenting with urinary symptoms.

Conclusion

Lichen sclerosus is an often underdiagnosed but important cause of urinary symptoms—particularly dysuria and recurrent UTIs—in pediatric patients. Raising awareness and ensuring early diagnosis not only improve clinical outcomes but also help avoid unnecessary invasive investigations. Therefore, pediatricians, nephrologists, and urologists should maintain a high index of suspicion for LS in children presenting with persistent dysuria, recurrent UTIs, or sterile pyuria. Optimal care requires a multidisciplinary approach, including dermatology consultation and management of associated voiding dysfunction. In conclusion, by ensuring timely diagnosis and intervention, clinicians can prevent unnecessary investigations and improve the long-term quality of life for pediatric patients with LS.

Author Contributions

AN and BA contributed to conceptualization. AN, BA, GP, and MD contributed to methodology. BA, GP, HÖG, MD, and AN contributed to data collection. AN and BA contributed to data analysis. BA contributed to writing—original draft preparation. BA, MD, and AN contributed to writing—review and editing. All authors contributed to the approval of the final draft. AN contributed to supervision.