It is widely acknowledged that LS exhibits a higher incidence in girls than boys [10], with a ratio of approximately 2.5/1 in our study. This gender disproportion might be attributed to a detection bias, as male children are often admitted for LS-related phimosis (14 out of 17 boys of our cohort—82.4% of boys underwent surgery for phimosis), whereas girls may remain asymptomatic initially, later developing typical lesions.

The mean age of our patients and the mean age at LS onset align with existing literature, where typically places the onset of LS in children between 3 and 14 years [11]. In our study, the mean age at LS onset was 7.4 ± 2.9 years in girls and 5.5 ± 2.5 years in boys, compared to a reported mean age of 8.6 years in males in the literature [12].

While previous studies reported a 10–17% ratio of family history in LS patients [10, 11], our findings differ, with only 8.3% of our patients reporting a family history of LS (1 boy—5.9% of males and 4 girls—9.3% of females).

Recent studies suggest that 15–34% of LS cases in adult women and 14% in girls coincide with allergies or autoimmune diseases.

In our population, we observed 1 patient with Hashimoto’s disease and 1 had celiac disease, and 4 (6.7%) had atopic dermatitis. Cutaneous comorbidities, such as atopic dermatitis, psoriasis, alopecia areata, and linear scleroderma, were more prevalent in females, while autoimmune systemic diseases, specifically Hashimoto’s thyroiditis and celiac disease, showed a higher prevalence among males. These findings underline the need for ongoing clinical monitoring in LS patients to assess treatment response, advise on long-term control [12, 13], and detect potential onset of other autoimmune and/or skin diseases.

In our study, erythema was the most common clinical sign (observed in 43 patients—78.3%), followed by paleness (17 patients—28.3%). Phimosis was the predominant clinical sign in boys, while skin erosions and purpura were rare (detected in 2 patients—3.3%). These data are in accordance with data reported in literature [12, 13].

Among females, the perianal region was involved in 18 patients (41.9%), without causing constipation. Small and large genital lips, vulvar vestibule, and clitoris were frequently affected. In males, the inner and outer leaflet of the foreskin, as well as the penis were the most commonly affected areas. LS affected the urethra in 1 male patient, and no extra-genital lesions were detected.

Symptom percentages mirrored literature findings [12], with pruritus observed in 25 girls (58.1% of females) and 2 boys (11.8% of males) while pain and burning sensations were reported only by females. LS commonly affects the anogenital region in girls, whereas boys often experience involvement of the glans, foreskin, and the urethra [14].

According to the guidelines from the “British Association of Dermatologists” (2018) [13], high-potency topical corticosteroids, particularly clobetasol propionate (CP) 0.05% ointment, are recommended as the first-line treatment for LS. The advised regimen is once daily for 3 months. Furthermore, the guidelines suggest that adults should continue using CP 0.05% for ongoing active LS disease, and there is insufficient evidence to recommend topical calcineurin inhibitors for LS patients [13].

In our study population, tacrolimus 0.1% ointment was well-tolerated by all patients, with 28 patients (46.7%) successfully using it once daily. Only 11 patients (18.3%) used mometasone furoate 0.05% cream as a topical steroid, and none required high-potency steroids like CP 0.05% Male children required chronic application of calcineurin inhibitors, but firstly they needed to use topical corticosteroids likely because phimosis typically responds better to this treatment [15]. Female children with LS usually required chronic use of a calcineurin inhibitor to settle inflammation, and less commonly, they needed to use topical corticosteroids.

The primary advantages of topical calcineurin inhibitors in a long-term treatment compared to conventional topical corticosteroids lie in their more selective mechanism of action, without the potential side effects associated with corticosteroids, such as skin atrophy [16, 17]. Given that LS is a chronic disease requiring prolonged treatment and that affected skin areas are often atrophic, a topical drug that induces skin atrophy may not be the optimal therapeutic choice.

Regarding safety, topical calcineurin inhibitors are generally well tolerated in our experience. Although they may be associated with mild-to-moderate and transient application-site reactions, such as pruritus and skin burning, these symptoms typically resolve with continued treatment, as reported in literature [18].

Based on our experience, we propose a flow-chart for the management of LS in pediatric patients, suggesting the use of mild-potency topical corticosteroids (TCS) for acute flares, especially in cases with skin erosions and severe itching, followed by the introduction of topical calcineurin inhibitors for long-term treatment (Fig. 1). In cases presenting with itching (NRS > 6), hyperkeratosis, and erosions, especially when a higher severity was observed, we elected to use topical corticosteroids, given the robust support for their efficacy in the existing literature [12]. Conversely, for milder cases characterized by erythema, paleness, or an Itch NRS score < 6, we adopted topical calcineurin inhibitors (TCI) as the primary treatment, noting beneficial effects.

A potential limitation of our study is the relatively small number of patients; further research is undoubtedly warranted to elucidate the efficacy of our protocol and the use of topical calcineurin inhibitors.