Pediatric Lichen Sclerosus: A Review of the Literature and Management Recommendations

Huyenlan Dinh, DO; Stephen M. Purcell, DO; Catherine Chung, MD; Andrea L. Zaenglein, MD


Lichen sclerosus is an uncommon, inflammatory disease of the skin and mucosa that can cause significant pruritus, pain, and scarring. There are two peaks of incidence: one in adult females, and the other in young girls. Early recognition and prompt management of the disorder is crucial in preventing long-lasting complications. This article reviews lichen sclerosus in children and provides evidence-based guidance for treatment in the pediatric population. J Clin Aesthet Dermatol. 2016;9(9):49–54


Lichen sclerosus (LS) is a chronic inflammatory disease of the skin and mucosa. It commonly develops in the anogenital region, but can affect any area. LS presents more frequently in females with two peak ages of presentation: prepubertal girls and postmenopausal women. A study of 327 patients with LS showed a mean age of onset of disease at 5.4 years in girls and 55.1 years in women.[1] The prevalence rate ranges between 1:70 to 1:1000 in women and 1:900 in children.[2],[3] Delayed diagnosis is not uncommon in girls with LS, with an average duration until diagnosis of 1 to 1.6 years.[4–6]

The pathogenesis of LS is unknown. Autoimmune factors have been investigated and autoantibodies to extracellular matrix protein 1 titers were found in 80 percent of affected patients.[7] Association with other autoimmune diseases has been reported. In a study of 350 women with LS, 21.5 percent had one or more autoimmune-related diseases, most commonly, autoimmune thyroiditis, vitiligo, alopecia areata, and pernicious anemia.[8]

Celiac disease has also been associated with LS.[9] In 30 prepubertal girls with anogenital LS, 6.6 percent had associated autoimmune diseases, such as vitiligo and alopecia areata.[10] Genetic, hormonal, environmental, and infectious factors have also been implicated as possible causes of this disease.[11–15]

Clinical Features

Presenting symptoms in girls include pain, pruritus, and a burning sensation along the perineal region. Dysuria and local spotty bleeding can result due to fissuring of the skin along the affected areas. A classic “figure 8” pattern is described, involving the labia minora, clitoral hood, and perianal region (Figure 1). Lesions initially are white, flat-topped papules, thin plaques, or commonly atrophic patches. Purpura is a hallmark feature of vulvar LS. Hyperpigmentation, erosions, and ulceration can result. Secondary constipation is also a common complication, occurring in 67 percent of girls with anogenital LS.[4] Young girls will withhold stooling due to the pain; subsequent management can be quite difficult, with habits and symptoms persisting even after effective treatment of the LS. Due to the nature of the symptoms, suspicion for child abuse can arise and may warrant further investigation when dealing with the pediatric population.[3]

In males, LS on the penis is called balanitis xerotica obliterans. The incidence has varied with some reporting 0.07 to up to 0.3 percent occurring in children as young as two years old and in adults, with the highest prevalence at ages 61 or older.[16],[17] Atrophic, shiny white, thin plaques usually involve the glans penis and can extend onto the shaft. Boys commonly present with associated phimosis. In a study of 1,178 boys with acquired phimosis, 40 percent were found to have LS on circumcision pathology.[18]

Extragenital LS can occur anywhere on the body, but typically involves the back, chest, and breasts (Figure 2). Oral mucosal involvement has also been reported and can mimic vitiligo early on.[19] Clinically, extragenital LS presents as white flat papules that coalesce into plaques. The color often has a shiny porcelain look and may be surrounded by an erythematous or violaceous halo (Figure 2). Scarring is common. Blaschkoid, segmental, and bullous types have been reported as well as overlap with cutaneous morphea. The lesions are mostly asymptomatic and can occur with or without genital involvement.[20]


Since the diagnosis of LS is usually clinical, biopsy is reserved for cases if there is a doubt in diagnosis, a suspicion for neoplastic change, resistance to adequate treatment, or atypical extragenital presentations. Histopathologically, well-developed lesions of LS show an atrophic epidermis, hyperkeratosis, edema in the papillary dermis with collagen homogenization, and an underlying lymphocytic infiltrate. This pattern is often referred to as “red, white, and blue” on low-power hematoxylin and eosin evaluation due to the eosinophilic hyperkeratosis (red), pale-staining papillary dermis (white), and basophilic lymphocytic infiltrate (blue). Follicular plugging is also a common feature (Figure 3 and Figure 4).[21]

Due to the chronicity of inflammation, progression of LS can obliterate the normal anatomic structures due to scarring. The disease can relapse and become a lifelong condition. A prospective case series followed 12 girls with prepubertal LS for 10 years until adolescence. The results showed that 25 percent had complete remission, whereas 75 percent remained symptomatic with clinical signs of the disease as adolescents.[22] Even though they were diagnosed early and received treatment, childhood onset LS can still be complicated by distortion of vulvar architecture. Other complications include a five percent lifetime risk of developing squamous cell carcinoma; however, this is usually seen in older adult patients who had longer duration of LS.[23] Vulvar melanoma has also been reported in a child with LS.[24]


The use of ultrapotent topical corticosteroids (UPTCS) has been the mainstay of treatment of LS in all age groups. Studies have shown that application of UPTCS led to improvement of symptoms in almost all patients.[1],[25] In pediatric LS, a case series showed successful treatment in childhood vulvar LS with topical corticosteroids (betamethasone dipropionate 0.05% ointment, diflorasone diacetate 0.05% ointment, or clobetasol propionate 0.05% ointment) twice daily for 6 to 8 weeks with minimal side effects observed.[26] Another study of 74 girls treated with potent or UPTCS demonstrated marked improvement, with 72 percent becoming symptom free after at least three months of topical treatment.1 Long-term follow up (4.7 years) in a retrospective review of 15 young girls showed that early aggressive treatment led to a better therapeutic response.[27] While mid-potency topical corticosteroids (TCS), such as triamcinolone acetonide or mometasone furoate, have been found to be effective in LS,[28],[29] most current recommendations do not support their use for first-line treatment.

Topical calcineurin inhibitors (TCIs), tacrolimus and pimecrolimus, are also used as adjunctive therapy for LS, but have not been shown as effective in initial treatment for LS both clinically and histologically.[30],[31] Several studies have shown the efficacy of using TCIs daily or twice daily in children and adults with partial to complete remission ranging from 6 weeks to 10 months of treatment.[32–36] After using TCIs after 16 weeks, complete remission was seen in only 43 percent of patients and partial remission was seen in 34 percent of patients.[36] Side effects of TCIs included stinging and burning. Concern for the use of TCIs stems from the intrinsic malignant potential that TCIs may increase the risk of SCC development in patients with LS especially with long-term use.[37],[38] Combining therapy with TCS and TCIs has been studied. Several authors reported remission in pediatric cases with LS after alternating clobetasol 0.05% ointment with tacrolimus ointment.[39] Clobetasol 0.05% ointment was used first and then tacrolimus ointment was bridged into the therapy regimen. As patients cleared, clobetasol ointment was discontinued all together and maintenance was achieved with tacrolimus ointment on weekends only. Clearance ranged from 4 to 156 weeks with an average time of 43.1 weeks.

Recent data emphasizes the importance of maintenance therapy for anogenital LS in both women and children to prevent long-term sequelae.[6],[40] These studies used low-to-mid potent TCS for maintenance. In a study involving 46 girls with prepubertal onset vulvar LS, patients were treated early with UPTCS daily and then decreased to a low-to-mid potency TCS for maintenance.[6] With this regimen, the patients were followed for a mean duration of 32.8 months. Of the 46 patients, 71.3 percent were adherent to the long-term treatment and 93.3 percent of those achieved complete disease suppression. None of the patients who stayed on the maintenance therapy with the low potency TCS experienced progression of scarring. The authors concluded that long-term maintenance with low- or mid-potency TCS was recommended until at least puberty.

Systemic therapy can be considered in refractory LS, or in generalized LS. Oral corticosteroids, methotrexate, acitretin, isotretinoin, cyclosporine, hydroxyurea, ceftriaxone, penicillin G, sulphasalazine, and vitamin A combined with vitamin E or D may be used for treatment-resistant LS.[41] However, the level of evidence and grade of the recommendations is very low due to limited numbers of patients and lack of clinical trials. Pulsed corticosteroids (methylprednisolone 1gm IV daily for three consecutive days monthly for 3 months) combined with low-dose methotrexate (15mg/wk), similar to morphea management, has been used in refractory extragenital LS.[42] Patients showed improvement after three months of treatment and they were treated for at least six months. Systemic retinoids did show some efficacy in the treatment of genital LS and can be considered if standard therapy for LS has failed. Acitretin dosed at 20 to 30mg/day for 16 weeks did show symptomatic improvement in patients.[43] Cyclosporine was used in patients with refractory vulvar LS with symptomatic improvement and decrease in erythema and erosions after one month of therapy.[44]

Recommendations for Management

Based on the data listed above, several groups created recommendations for topical treatment of LS. In 2010, the British Association of Dermatologists recommended that patients with LS should be managed with UPTCS as first-line treatment in newly diagnosed LS with an alternating schedule every four weeks; however, no long-term maintenance regimen was included.37 In 2015, Ellis and Fischer6 recommended both short-term treatment and long-term maintenance therapy for prepubertal patients with LS. They endorsed starting with daily use of a superpotent or potent TCS, depending on the initial severity. Patients should be evaluated at 4 weeks and continue monotherapy until all symptoms and signs resolve. Every six weeks, patients should be monitored for remission and side effects. If symptoms resolve, TCS potency can be gradually reduced and maintenance therapy started. A combination therapy that included hydrocortisone 1% ointment and methylprednisolone aceponate 0.1% ointment was recommended. Hydrocortisone 1% ointment was used daily and methylprednisolone aceponate 0.1% ointment used on the weekends. Patients were re-evaluated three months after, then every six months after while on maintenance therapy. If the disease was controlled for two years without side effects, it was recommended that patients be monitored yearly. Maintenance therapy is to be continued at least until patients reach puberty. It should be noted that the authors in this study did not incorporate TCIs into their regimen.

The European Academy of Dermatology and Venerology recommended incorporating TCS and TCIs.41 They recommended initiating treatment with clobetasol propionate 0.05% ointment or cream once or twice daily for three months with possible reduction in application frequency after one month. Depending on the patient, TCS may only need to be used once or twice a month, up to two to three times a week. Mometasone furoate 0.1% ointment was the recommended TCS for proactive maintenance therapy with twice-weekly application. The first follow-up should be at three months and if the diseases remain uncomplicated, follow-up visits can be done at six months. Moisturizers and silk underwear were also recommended, as decreasing friction was associated with fewer symptoms. The authors did not provide a specific guideline or recommendation for TCIs, but they did note that TCIs may be an effective alternative to strong TCS and could be used as maintenance therapy.

Taking into account the available data and various recommendations, the authors suggest a management plan for pediatric LS with combination therapy (Table 1 ).


Pediatric treatment of LS can be complex and should be individualized based on the patient’s degree of symptoms. Using UPTCS initially can halt or slow the degree of inflammation. Maintenance should be obtained with the use of TCIs or low potency TCS. Regular follow up is needed to assess the degree of inflammation and symptoms. Pediatric LS should be treated promptly to reduce symptoms and decrease the risk of vulvar scarring and distortion. Long-term follow up is needed as recurrences are not uncommon.


1. Maronn ML, Esterly NB. Constipation as a feature of anogenital lichen sclerosus in children. 2005;115(2):e230–e232.

2. Goldstein AT, Marinoff SC, Christopher K, Srodon M. Prevalence of vulvar lichen sclerosus in a general gynecology practice. J Reprod Med. 2005;50:477–480.

3. Powell J, Wojnarowska F. Childhood vulvar lichen sclerosus: an increasingly common problem. J Am Acad Dermatol. 2001;44:803–806.

4. Maronn ML, Esterly NB. Constipation as a feature of anogenital lichen sclerosus in children. Pediatrics. 2005;115:230–232.

5. 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(3):289–292.

6. Elis E, Fischer G. Prepuberal-onset vulvar lichen sclerosus: the importance of maintenance therapy in long-term outcomes. Pediatr Dermatol. 2015;32(4):461–467.

7. Oyama N, Chan I, Neill SM, et al. Development of antigen-specific ELISA for circulating autoantibodies to extracellular matrix protein 1 in lichen sclerosus. J Clin Invest. 2004;113(11):1550–1559.

8. Meyrick Thomas RH, Ridley CM, McGibbon DH, Black MM. Lichen sclerosus et atrophicus and autoimmunity—a study of 350 women. Br J Dermatol. 1988;118(1):41–46.

9. Jacobs L, Gilliam A, Khavari N, Bass D. Association between lichen sclerosus and celiac disease: a report of three pediatric cases. Pediatr Dermatol. 2014;31(6):e128–e131.

10. Powell J, Wojnarowska F, Winsey S, et al. Lichen sclerosus premenarche: autoimmunity and immunogenetics. Br J Dermatol. 2000;142(3):481–484.

11. Eisendle K, Grabner T, Kutzner H, Zelger B. Possible role of Borrelia burgdorferi sensu lato infection in lichen sclerosus. Arch Dermatol. 2008;144(5):591–598.

12. Aidé S, Lattario FR, Almeida G, et al. Epstein-Barr virus and human papillomavirus infection in vulvar lichen sclerosus. J Low Genit Tract Dis. 2010;14(4):319–322.

13. Günthert AR, Faber M, Knappe G, et al. Early onset vulvar lichen sclerosus in premenopausal women and oral contraceptives. Eur J Obstet Gynecol Reprod Biol. 2008;137(1):56–60.

14. Sherman V, McPherson T, Baldo M, et al. The high rate of familial lichen sclerosus suggests a genetic contribution: an observational cohort study. J Eur Acad Dermatol Venereol. 2010;24(9):1031–1034.

15. Bunker CB. Male genital lichen sclerosus and tacrolimus. Br J Dermatol. 2007;157(5):1079–1080.

16. Kizer WS, Prarie T, Morey AF. Balanitis xerotica obliterans: epidemiologic distribution in an equal access health care system. South Med J. 2003;96(1):9–11.

17. Nelson DM, Peterson AC. Lichen sclerosus: epidemiological distribution in an equal access health care system. J Urol. 2011;185(2):522–525.

18. Kiss A, Király L, Kutasy B, Merz M. High incidence of balanitis xerotica obliterans in boys with phimosis: prospective 10-year study. Pediatr Dermatol. 2005;22(4):305–308.

19. Attili VR, Attili SK. Lichen sclerosus of lips: a clinical and histopathologic study of 27 cases. Int J Dermatol. 2010;49(5):520–525.

20. Bergstrom K, Mengden, S, Kamino, H, Ramsay D. Extragenital lichen sclerosus et atrophicus. Dermatology Online Journal. 2008;14(5). uc/item/3g0275rm. Accessed on June 1, 2016.

21. Winfield H, Jaworsky C. Connective tissue diseases. In: Elder D, Elenitsas R, Johnson B, et al. Lever’s Histopathology of the Skin. 10th edition, Philadelphia, PA: Lippincott Williams & Wilkins; 2009; 303–307.

22. Smith SD, Fischer G. Childhood onset vulvar lichen sclerosus does not resolve at puberty: a prospective case series. Pediatr Dermatol. 2009; 26(6):725–729.

23. Jones RW, Sadler L, Grant S, et al. Clinically identifying women with vulvar lichen sclerosus at increased risk of squamous cell carcinoma: a case-control study. J Reprod Med. 2004;49(10):808–811.

24. La Spina M, Meli MC, De Pasquale R, et al. Vulvar melanoma associated with lichen sclerosus in a child: case report and literature review. Pediatr Dermatol. 2016;33(3):e190–e194.

25. Dalziel KL, Millard PR, Wojnarowska F. The treatment of vulval lichen sclerosus with a very potent topical steroid (clobetasol propionate 0.05%) cream. Br J Dermatol. 1991;124(5):461–464.

26. Garzon M, Paller A. Ultrapotent topical corticosteroid treatment of childhood genital lichen sclerosus. Arch Dermatol. 1999;135(5): 525–528.

27. Patrizi A, Gurioli C, Medri M, Neri I. Childhood lichen sclerosus: a long-term follow-up. Pediatr Dermatol. 2010;27(1):101–103.

28. Cattaneo A, De Magnis A, Botti E, et al. Topical mometasone furoate for vulvar lichen sclerosus. J Reprod Med. 2003;48(6):444–448.

29. LeFevre C, Hoffstetter S, Meyer S, Gavard J. Management of lichen sclerosus with triamcinolone ointment: effectiveness in reduction of patient symptom scores. J Low Genit Tract Dis. 2011;15(3):205–209.

30. Funaro D, Lovett A, Leroux N, Powell J. A double-blind, randomized prospective study evaluating topical clobetasol propionate 0.05% versus topical tacrolimus 0.1% in patients with vulvar lichen sclerosus. J Am Acad Dermatol. 2014;71(1):84–91.

31. Goldstein AT, Creasey A, Pfau R, et al. A double-blind, randomized controlled trial of clobetasol versus pimecrolimus in patients with vulvar lichen sclerosus. J Am Acad Dermatol. 2011;64(6):e99–e104.

32. Assmann T, Becker-Wegerich P, Grewe M, et al. Tacrolimus ointment for the treatment of vulvar lichen sclerosus. J Am Acad Dermatol. 2003;48(6):935–937.

33. Ginarte M, Toribio J. Vulvar lichen sclerosus successfully treated with topical tacrolimus. Eur J Obstet Gynecol Reprod Biol. 2005;123(1):123–124.

34. Kunstfeld R, Kirnbauer R, Stingl G, et al. Successful treatment of vulvar lichen sclerosus with topical tacrolimus. Arch Dermatol. 2003;139:850–852.

35. Böhm M, Frieling U, Luger TA, et al. Successful treatment of anogenital lichen sclerosus with topical tacrolimus. Arch Dermatol. 2003;139:922–924.

36. Hengge UR, Krause W, Hofmann H, Stadler R, et al. Multicenter, Phase II trial on the safety and efficacy of topical tacrolimus ointment for the treatment of lichen sclerosus. Br J Dermatol. 2006;155(5):1021–1028.

37. Neill SM, Lewis FM, Tatnall FM, Cox NH, British Association of Dermatologists. British Association of Dermatologists’ guidelines for the management of lichen sclerosus 2010. Br J Dermatol. 2010;163(4):672–682.

38. Bunker CB, Neill SM, Staughton RCD. Topical tacrolimus, genital lichen sclerosus and risk of squamous cell carcinoma. Arch Dermatol. 2004;140:1169.

39. Anderson K, Ascanio N, Kinney M, et al. A retrospective analysis of pediatric patients with lichen sclerosus treated with a standard protocol of class 1 topical corticosteroid and topical calcineurin inhibitor. J Dermatol Treat. 2016;27(1):64–66.

40. Lee A, Bradford J, Fischer G. Long-term management of adult vulvar lichen sclerosus: a prospective cohort study of 507 women. JAMA Dermatol. 2015;151(10):1061–1067.

41. Kirtschig G, Cooper S, Aberer W, et al. Evidence-based (S3) guideline on (anogenital) lichen sclerosus. J Eur Acad Dermatol Venereol. 2015;29(10):e1–e43.

42. Kreuter A, Tigges C, Gaifullina R, et al. Pulsed high-dosed corticosteroids combined with low-dose methotrexate treatment in patients with refractory generalized extragenital lichen sclerosus. Arch Dermatol. 2009;145:1303–1308.

43. Bousema MT, Romppanen U, Geiger JM, et al. Acitretin in the treatment of severe lichen sclerosus et atrophicus of the vulva: a double-blind, placebo-controlled study. J Am Acad Dermatol 1994;30: 225–231.

44. Bulbul BE, Turan H, Tunali S, et al. Open-label trial of cyclosporine for vulvar lichen sclerosus. J Am Acad Dermatol. 2007;57:276–278.