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Year : 2021  |  Volume : 22  |  Issue : 2  |  Page : 131-135

Vulvar lichen sclerosus in children: A prospective study over a 3 year period

1 Dr Paruchuri Rajaram Memorial Skin, Hair and Laser Centre, Guntur, Andhra Pradesh, India
2 Hanuma Skin Center, Guntur, Andhra Pradesh, India

Date of Submission03-Oct-2020
Date of Decision29-Nov-2020
Date of Acceptance03-Jan-2021
Date of Web Publication31-Mar-2021

Correspondence Address:
Kavitha Athota
Dr. Paruchuri Rajaram Memorial Skin, Hair and Laser Centre, Near Kugler Hospital Road, 12-13-1, Kothapet, Guntur - 522 001, Andhra Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijpd.ijpd_153_20

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Context: Vulvar lichen sclerosus (VLS) is a chronic inflammatory disease affecting prepubertal girls and postmenopausal women. In prepubertal girls, it causes anxiety in parents and affects quality of life considerably. There is a paucity of data about VLS in children from India. Aims: We aimed to study the demographic and clinical profile of VLS in children. Settings and Design: It is a prospective study over a period of 3 years from October 2017 to September 2020. Materials and Methods: All the children up to the age of 15 years with VLS, attending to our outpatient department between October 2017 and September 2019, were enrolled in the study after taking informed consent from the parents/guardian. The age, duration, clinical features, and comorbidities were noted. Biopsy was done only in clinically doubtful cases. All the cases were treated with topical clobetasol 0.05% ointment once daily for 3 months, and tacrolimus 0.03% or pimecrolimus 1% ointment was given as maintenance therapy. Cases were followed up for a period of 6 months–3 years. Results: Twenty-five girls presented with VLS during the study period. The mean age was 8.44 years, and the mean duration at presentation was 1.14 years. Pruritus was present in 14 (56%); constipation and dysuria were noted in 2 each. Classical figure-of-eight appearance was observed in only 4 cases; atrophy and erythema were noted in 11 cases and 3 cases, respectively. All symptoms improved in 3–4 weeks after treatment and depigmentation completely subsided in 8 cases by 3 months. Limitations: The diagnosis of VLS was made clinically in most cases. Dermoscopy was not done. Biopsy was done in only clinically doubtful cases. Conclusions: VLS in children needs special attention as it causes anxiety in parents thinking it as vitiligo. Pruritus is the common symptom. Ultrapotent topical steroid helps in the management. It may not resolve completely at puberty.

Keywords: Children, Lichen sclerosus, Vulva

How to cite this article:
Athota K, Kolalapudi SA. Vulvar lichen sclerosus in children: A prospective study over a 3 year period. Indian J Paediatr Dermatol 2021;22:131-5

How to cite this URL:
Athota K, Kolalapudi SA. Vulvar lichen sclerosus in children: A prospective study over a 3 year period. Indian J Paediatr Dermatol [serial online] 2021 [cited 2021 Dec 2];22:131-5. Available from: https://www.ijpd.in/text.asp?2021/22/2/131/312817

  Introduction Top

Vulvar lichen sclerosus (VLS) in children is a heterogeneous disease with remarkable effect on quality of life. Most of the cases are thought of as vitiligo, therefore, causing anxiety among the parents. VLS has been reported in the anogenital area, with a prevalence of 1 in 900 girls.[1] Pruritus and burning are the main presenting symptoms in adults, but in children constipation, dysuria and depigmentation may be the presenting features.[2] Trauma, infection, genetic factors, hormonal factors, and urinary incontinence are implicated in the pathogenesis of lichen sclerosus (LS).[2],[3],[4] Quality of life is impaired in VLS in children, and Lagerstedt reported impaired quality of life in 67% of children with LS.[5] A systematic review reported 37 studies with VLS in children from the world literature, but there was none from Indian literature.[6] We assessed the VLS in children as there is a paucity of data from India.

  Materials and Methods Top

Patients attending to our outpatient department, with VLS during October 2017 to September 2019, were included in the study and were followed up to September 2020. Children up to the age of 15 years were included in the study. Ethics committee has approved this study (GMC/IEC/122/2017). Consent was taken from the parents/guardian. Complete demographic data (age, sex, occupation, family history, etc.), presenting symptoms (depigmentation, pruritus, constipation, dysuria, etc.), and clinical findings (extent, atrophy, induration, ecchymosis, purpura, ulceration, etc.) were noted. The diagnosis was made based on the classical clinical features, and biopsy was done in 10 doubtful cases with the consent of parents. Patients were followed up for a period of 6 months–3 years. Associated autoimmune conditions such as hypo- or hyperthyroidism, vitiligo, and alopecia were noted. Most of the cases presented with depigmentation as the main presenting symptom, and vitiligo was the major differential diagnosis. Vitiligo is asymptomatic depigmentation without any alteration in skin texture which helped in differentiating from LS.

In all cases, clobetasol propionate 0.05% ointment was given daily for 1 month, alternate day for the next month, and weekly twice in the 3rd month along with an emollient. Topical tacrolimus 0.03% was added at night during the 2nd month. After 3 months, tacrolimus was continued as maintenance therapy and topical steroid was restarted in only symptomatic flare-up of the disease.

  Results Top

Twenty-five girls presented with VLS during the study period. This was about 13.4% of the total VLS in females we observed during this period (25/186). All of them were between 3 and 14 years, with a mean age of 8.44 years. Duration at the time of presentation was varying from 3 months to 3 years, with a mean of 1.14 years. The clinical profiles are shown in [Table 1]. All 25 presented with depigmentation (100%) [Figure 1]. Pruritus was present in 14 (56%); constipation and dysuria were noted in 2 each. Labia alone were involved in 16 cases, and the lesions extended to groins in five cases. Perianal area was involved in 4 cases, giving rise to classical figure-of-eight appearance [Figure 2]. Atrophy was noted in 11 cases and ecchymosis was noted in 3 cases [Figure 3]. Biopsies were done in ten cases. Histopathology showed atrophic epidermis, superficial and mid-perivascular infiltrate of lymphocytes, plasma cells, and histiocytes with dilated capillaries in the dermis with collagen homogenization [Figure 4].
Table 1: Clinical profile of 25 children with vulval lichen sclerosus

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Figure 1: Vulvar lichen sclerosus presenting as depigmentation

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Figure 2: Figure-of-eight appearance

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Figure 3: Vulvar lichen sclerosus with ecchymotic spots

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Figure 4: Biopsy showing superficial and mid-perivascular infiltrate of lymphocytes, plasma cells, and histiocytes with dilated capillaries throughout dermis. Stratum corneum shows mild thickening with lamellar orthokeratosis (a: ×40 magnification) (b: ×100 magnification)

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VLS was noted in two pairs of siblings. There was no family history of VLS in any of the cases. Hypothyroidism was noted in four cases; vitiligo and alopecia areata were seen in one case each. Eleven cases attained menarche during the study period, and all of them continued to have VLS in the postpubertal period. In all symptomatic cases, symptoms improved by 3–4 weeks and 14 patients had started improving in depigmentation by 4–6 weeks of topical steroid therapy, and eight had improved completely at the end of 3 months. One of these eight had recurred after 1 year. The other 11 had shown mild-to-moderate improvement in depigmentation even after 6–12 months of treatment. Seven complained mild burning sensation with tacrolimus ointment and they were given pimecrolimus 1% ointment, which was tolerated well.

  Discussion Top

Vulvar LS has two peaks, prepubertal and postmenopausal. The prevalence of VLS in prepubertal age was about 1 in 900 girls amounting to 15% of total vulvar LS.[7] Various studies reported 4%–12% of VLS in children.[8],[9],[10] In our study, VLS in children was about 13.4%, which was slightly higher than the other studies across the world. The mean age was 8.44 years (range: 3–14 years), and the youngest patient was of 3 years old. The mean duration at the time of presentation was 15 months (range: 3–36 months). VLS is rare in young children <2 years of age because of maternal estrogen protection.[11]

All of them presented for depigmentation only. The symptoms noted at presentation were pruritus (14 patients, 56%) and constipation and dysuria (2 each, 8% each). Pruritus was the main associated symptom as reported in other studies.[1],[2],[10] Twenty-eight percent were asymptomatic in our group similar to Cooper's study (27.7%, 22/72), compared to 7% by Powell et al.[1],[10] Atrophy was noted in 44% of our study group, compared to 35% by Cooper et al. Ecchymosis was noted in 12%, compared to 15%–20% in other studies.[1],[10] Scarring and architectural changes were noted in 20%–50% of the cases in different series.[12],[13] Purpura was more frequent in childhood VLS in Copper's study (34%), however, purpura and ecchymosis were seen in only 3 of our cases (12%). Vaginal stenosis, which was more frequent in adult and old age VLS was not seen in our childhood group.

Family history of VLS was reported in 23%–36% in other studies, but we did not observe any family history of LS, either in males or female family members.[1],[10] However, we observed two pairs of siblings with VLS, and even in them, we did not find any positive family history. This could be because of lack of awareness among parents about the symptoms and signs of the disease entity. The occurrence in siblings was noted in other studies also indicating genetic susceptibility, and it should be further explored.[1]

Sexual abuse and VLS can coexist, and sometimes, abuse can trigger VLS through koebnerization.[14],[15] However, we could not elicit or did not suspect sexual abuse in any of our 25 cases. Sexual abuse in children involves hymen in contrast to VLS, where hymen is not affected.[16] It is important to suspect and identify sexual abuse in childhood VLS.

VLS is associated with autoimmune disease and higher incidence of antibodies.[2],[3],[17] Thyroid disease, celiac disease, rheumatoid arthritis, alopecia areata, and psoriasis were observed in some studies, and we noted hypothyroidism in 4 cases, vitiligo and alopecia areata in 1 patient each. Children with VLS and autoimmune disease had a strong HLA association than their adult counterparts, but HLA typing could not be done in our study.[18]

Vulvar squamous cell carcinoma (VSCC) in VLS is uncommon in children, compared to old age. However, Cario et al. reported VSCC at the age of 18 in a long-standing VLS and Wallace reported in two persons at the age of 25–35 years, who had VLS since childhood.[19],[20] Our follow-up was limited to a maximum of 3 years in some cases, and we did not observe any vulval carcinoma in these children.

Majority of the studies reported that VLS in children does not resolve at puberty.[1],[6] In majority of the children, symptoms may improve, but residual signs persist for longer time. Casey et al. reported residual signs in 75% of their study group at a follow-up of 4 years.[21] In our study also, 11 children attained menarche and continued to have VLS during postpubertal period, and there was no change in the course of the disease.

Ultrapotent topical corticosteroids improve the long-term outcome of VLS, and maintenance therapy with tacrolimus is promising in children.[6],[21] Fourteen (56%) patients improved by 3 months, but Casey et al. reported improvement in 90.3% cases at the end of 3 months. This could be because of shorter period of daily clobetasol use (1 month), in our group, compared to 3 months of daily use by Casey et al. However, all symptomatic patients improved completely at the end of 3–4 weeks. Ultrapotent topical steroid like clobetasol 0.05% is more effective compared to medium- and low-potent steroids.[21]

Nocturnal enuresis and urinary incontinence, causing wet skin, thus causing increased permeability to irritants, can trigger VLS[22],[23] and may result in treatment failure.[2] Two of our patients had dysuria and improved with topical clobetasol, and there was no recurrence of urinary symptoms in the follow-up period.


The diagnosis of VLS was made clinically in most cases. Biopsy was done in only doubtful cases. Dermoscopy forms an important part in diagnosing VLS and also in differentiating from vitiligo. In our study, we could not do dermoscopy. In childhood VLS where biopsy may be difficult, and parents may not give consent, dermoscopy plays an important role.

  Conclusions Top

VLS in children needs special attention as it causes anxiety in parents thinking of vitiligo. It may not resolve completely at puberty. Prognosis is better, and occurrence of squamous cell carcinoma is rare in children compared to old age. Early diagnosis in prepubertal girls helps in preventing long-term sequelae. Ultrapotent corticosteroids are the mainstay in the treatment, and tacrolimus or pimecrolimus can be used for maintenance therapy.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Powell J, Wojnarowska F. Childhood vulvar lichen sclerosus: An increasingly common problem. J Am Acad Dermatol 2001;44:803-6.  Back to cited text no. 1
Ismail D, Owen CM. Paediatric vulval lichen sclerosus: A retrospective study. Clin Exp Dermatol 2019;44:753-8.  Back to cited text no. 2
Fistarol SK, Itin PH. Diagnosis and treatment of lichen sclerosus: An update. Am J Clin Dermatol 2013;14:27-47.  Back to cited text no. 3
Virgili A, Borghi A, Toni G, Minghetti S, Corazza M. Prospective clinical and epidemiologic study of vulvar lichen sclerosus: Analysis of prevalence and severity of clinical features, together with historical and demographic associations. Dermatology 2014;228:145-51.  Back to cited text no. 4
Lagerstedt M, Karvinen K, Joki-Erkkilä M, Huotari-Orava R, Snellman E, Laasanen SL. Childhood lichen sclerosus--a challenge for clinicians. Pediatr Dermatol 2013;30:444-50.  Back to cited text no. 5
Morrel B, van Eersel R, Burger CW, Bramer WM, Ten Kate-Booij MJ, van der Avoort IAM, et al. The long-term clinical consequences of juvenile vulvar lichen sclerosus: A systematic review. J Am Acad Dermatol 2020;82:469-77.  Back to cited text no. 6
Goldstein AT, Marinoff SC, Christopher K, Srodon M. Prevalence of vulvar lichen sclerosus in a general gynecology practice. J Reprod Med 2005;50:477-80.  Back to cited text no. 7
Bleeker MC, Visser PJ, Overbeek LI, van Beurden M, Berkhof J. Lichen sclerosus: Incidence and risk of vulvar squamous cell carcinoma. Cancer Epidemiol Biomarkers Prev 2016;25:1224-30.  Back to cited text no. 8
Meyrick Thomas RH, Kennedy CT. The development of lichen sclerosus et atrophicus in monozygotic twin girls. Br J Dermatol 1986;114:377-9.  Back to cited text no. 9
Cooper SM, Gao XH, Powell JJ, Wojnarowska F. Does treatment of vulvar lichen sclerosus influence its prognosis? Arch Dermatol 2004;140:702-6.  Back to cited text no. 10
Warrington SA, de San Lazaro C. Lichen sclerosus et atrophicus and sexual abuse. Arch Dis Child 1996;75:512-6.  Back to cited text no. 11
Smith SD, Fischer G. Childhood onset vulvar lichen sclerosusdoes not resolve at puberty: A prospective case series. Pediatr Dermatol 2009;26:725-9.  Back to cited text no. 12
Nerantzoulis I, Grigoriadis T, Michala L. Genital lichen sclerosus in childhood and adolescence-a retrospective case series of 15 patients: Early diagnosis is crucial to avoid long-term sequelae. Eur J Pediatr 2017;176:1429-32.  Back to cited text no. 13
Powell JJ, Wojnarowska F. Lichen sclerosus. Lancet 1999;353:1777-83.  Back to cited text no. 14
Priestley BL, Bleehen SS. Lichen sclerosus and sexual abuse. Arch Dis Child 1990;65:335.  Back to cited text no. 15
Fischer GO. Vulval disease in pre-pubertal girls. Australas J Dermatol 2001;42:225-34.  Back to cited text no. 16
Kreuter A, Kryvosheyeva Y, Terras S, Moritz R, Möllenhoff K, Altmeyer P, et al. Association of autoimmune diseases with lichen sclerosus in 532 male and female patients. Acta Derm Venereol 2013;93:238-41.  Back to cited text no. 17
Powell J, Wojnarowska F, Winsey S, Marren P, Welsh K. Lichen sclerosus premenarche: Autoimmunity and immunogenetics. Br J Dermatol 2000;142:481-4.  Back to cited text no. 18
Cario GM, House MJ, Paradinas FJ. Squamous cell carcinoma of the vulva in association with mixed vulvar dystrophy in an 18-year-old girl. Case report. Br J Obstet Gynaecol 1984;91:87-90.  Back to cited text no. 19
Wallace HJ. Lichen sclerosus et atrophicus. Trans St Johns Hosp Dermatol Soc 1971;57:9-30.  Back to cited text no. 20
Casey GA, Cooper SM, Powell JJ. Treatment of vulvar lichen sclerosus with topical corticosteroids in children: A study of 72 children. Clin Exp Dermatol 2015;40:289-92.  Back to cited text no. 21
Al-Niaimi F, Lyon C. Peristomal lichen sclerosus: The role of occlusion and urine exposure? Br J Dermatol 2013;168:643-6.  Back to cited text no. 22
Gupta S, Malhotra AK, Ajith C. Lichen sclerosus: Role of occlusion of the genital skin in the pathogenesis. Indian J Dermatol Venereol Leprol 2010;76:56-8.  Back to cited text no. 23
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