Juvenile lichen sclerosus: A loss of innocence
By Warren R. Heymann, MD
August 19, 2020
Vol. 2, No. 33
Juvenile lichen sclerosus (LS) is a difficult disorder — symptomatically, emotionally, diagnostically, and therapeutically. During my residency it was “common knowledge” that juvenile LS would abate by adolescence. I am always curious how such perceptions become dogma, only to be retracted by future analysis.
Vulvar LS has a bimodal distribution occurring in childhood and in postmenopausal women; 5% to 15% of cases of LS occur in prepubertal girls with an estimated prevalence of 1 in 900. The average age of LS onset in girls is 4 to 6 years, however there is often a diagnostic delay of 1 or 2 years as other disorders are considered — contact dermatitis, vitiligo, lichen planus, morphea, infections, and child abuse. Although often asymptomatic, patients may experience anogenital pruritus, soreness, constipation, and dysuria. (1)
Morell et al conducted a systematic review of 37 studies yielding information on long-term consequences of vulvar LS, of which 13 were cohort studies and 24 case reports or series. These publications revealed that signs and symptoms persist after puberty and beyond, scarring and permanent architectural changes occur, treatment is effective with regard to symptoms, and long-term quality of life is affected. A possible relationship with risk of malignancy was suggested. The authors concluded there is low-level evidence suggesting long-term repercussions of juvenile vulvar LS. (2)
Ismael and Owen performed a retrospective analysis of 26 pediatric patients with vulvar LS. The median age at onset of symptoms was 5 years (range 2–8.5 years). Potential etiological factors for the development of vulvar LS were identified, including family history, trauma, autoimmune disease, and hormonal factors. A significant proportion of patients had a history of urinary tract symptoms, including incontinence and urinary tract infection. Most patients responded well to a standard course of induction topical therapy followed by maintenance therapy, but some, including three patients with ongoing urinary incontinence and three postpubertal patients, continued to have active disease. The authors concluded that a thorough evaluation is warranted in patients with vulvar LS so that potential predisposing factors and comorbidities can be identified and managed. Urinary incontinence may be implicated in the development of pediatric vulvar LS and addressing this may result in adequate disease control. Additionally, pediatric vulvar LS can persist through puberty, thus long-term follow-up is advised. (3)
Etiologically, LS has long been known to be associated with a variety of autoimmune disorders such as thyroid disease and celiac disease. The overwhelming prevalence in girls and postmenopausal women suggests hormonal influence. The association of cases of Turner syndrome, and its hypoestrogenic state, support this hypothesis. (1,3)
Assuming that pediatric vulvar LS may persist into adulthood, then concern for complications such as obliteration of the labia minora and clitoris, narrowing of the vaginal introitus, and an increased risk of squamous cell carcinoma (SCC), is warranted, thereby mandating ongoing treatment and vigilance. Aside from SCCs, genital lentigines and melanocytic nevi with associated LS may show features that mimic MM. (4) Despite potential mimicry, La Spina et al reported the case of an 11-year-old girl with a synchronous vulvar melanoma and LS. In their review, they identified 5 other cases of pediatric vulvar melanoma, all being associated with LS (there were 4 such cases in adults). (5) This association raises the conundrum — does the immunologic reaction to melanoma induce LS, or does the chronic inflammation of LS induce MM?
Therapeutically, there is little controversy that ultrapotent topical steroids are the treatment of choice. Calcineurin inhibitors are valuable, although not quite as effective, and, theoretically, could increase the risk of malignancy, although this is not a practical concern. Notay et al reported the case of a 6-year-old girl with vulvar LS who developed Cushing syndrome (moon facies, plethora, and weight gain) after 8 weeks of clobetasol. (6) Anderson et al performed a retrospective case series of 14 pediatric females between 2 and 10 years of age with LS treated with clobetasol 0.05% topical ointment and systematically bridged to tacrolimus 0.1% topical ointment. The authors concluded that using clobetasol to induce remission and tacrolimus to maintain remission can be used for LS in pediatric females. Their suggested regimen may minimize adverse effects associated with long-term, ultrapotent corticosteroid use and reduce the risk of changes to genital architecture secondary to LS itself. (7)
In conclusion, even though juvenile LS may be asymptomatic and resolve on its own, many cases may be symptomatic, persist, and have complications that mirror those of adult onset LS.
Point to Remember: Childhood vulvar lichen sclerosus may persist, causing complications usually associated with adult disease. Patients may be managed with ultrapotent topical steroids and calcineurin inhibitors, but long-term follow-up is essential.
Our expert's viewpoint
Libby Edwards, MD
Adjunct Clinical Associate Professor of Dermatology, University of North Carolina
Dr. Heymann’s insightful discussion of childhood lichen sclerosus addresses an important and generally ignored topic. As a former pediatrician, I never heard of this disease until I began my dermatology residency. I suspect that the prevalence is considerably higher than realized. I am continually surprised at the frequency of the appearance of this “rare” disease in my pre-existing pediatric dermatology patients.
Dr. Heymann is absolutely correct that many of these girls do not outgrow their lichen sclerosus. I suspect that symptoms improve because estrogen appears, improving the natural hypo-estrogenic atrophic skin changes. However, skin changes of lichen sclerosus persist in many if not most girls after puberty. Just because an adolescent stops medication and remains asymptomatic does not mean that the disease is in remission, without risk of silent scarring and eventual malignancy. The ongoing, careful use of corticosteroids in adults has been shown in a large prospective observational study to minimize scarring and the development of squamous cell carcinoma, so its use should not be avoided in girls (JAMA Dermatol. 2015;151:1061-7). If a child has absolutely no signs of lichen sclerosus at puberty, I give her the option of continuing medication, or stopping it and seeing me quarterly for 18 months to monitor for recurrence. There is no known protocol or data, and we do not know the risk of recurrence at menopause in girls who clear, or during the hypo-esotrogenic state associated with delivery of a child and breastfeeding.
Dr Heymann discusses the literature that addresses concerns of chronic topical corticosteroids in these children with lichen sclerosus. I absolutely agree that these reports should not lead providers to avoid chronic topical corticosteroids. Providers have years of experience finding topical corticosteroids to be effective and safe on the vulva of children when used appropriately. Although open label studies have shown calcineurin inhibitors to be useful for lichen sclerosus, providers generally find them to be less effective, more expensive, and often not covered by insurance. Other difficulties in using the calcineurin inhibitors include the black boxed warning for squamous cell carcinoma (which already is known to occur in 3-5% of women with lichen sclerosus) and, most importantly, they are often not tolerated due to burning with application. Certainly, improvement of symptoms is delayed with calcineurin inhibitors compared to topical corticosteroids.
Dr Heymann also alludes to a manuscript reporting the development of clinical iatrogenic Cushing’s syndrome following two months of clobetasol applied to the vulva of a child in which a 60-gram tube was dispensed. This is an extremely important reminder to providers both to demonstrate the tiny amount of medication needed for all steroid-responsive dermatoses, to limit the amount of medication provided, and write for no refills. One does not educate in terms of “fingertip units or pea-sized amounts” on the vulva. Far, far less is needed. A 15-gram tube of medication should treat the vulva for a year when properly used, and a super potent preparation is generally only needed for a few months to reverse skin changes. Then, a super potent preparation can be used only three times a week, or a midpotency corticosteroid used daily controls the condition. This should not cause systemic or topical side effects.
Although these issues — the occurrence of lichen sclerosus in children, the usefulness of a super potent corticosteroid in reversing the skin changes, the need for chronic therapy, and the persistence of lichen sclerosus following puberty — are well documented, children with lichen sclerosus are distressingly often under-recognized and mismanaged.
Editor’s note: This commentary was published in an abridged form as “A Clinician’s Perspective” in the February 2020 issue of the Journal of the American Academy of Dermatology.
Lagerstedt M, Karvinen K, B.M., Joki-Erkkilä M, Huotari-Orava R, et al. Childhood lichen sclerosus — A challenge for clinicians. Pediatr Dermatol 2013; 4: 444-450.
Morell B, van Eersel R, Burger CW, Bramer WM, et al. The long-term clinical consequences of juvenile vulvar lichen sclerosus, a systematic review. J Am Acad Dermatol 2019; Aug 19 [Epub ahead of print]
Ismail D, Owen CM. Paediatric vulval lichen sclerosus: A retrospective study. Clin Exp Dermatol 2019; 44: 753-758.
El Shabrawi-Caelen L, Soyer HP, Schaeppi H, Cerroni L, et al. Genital lentigines and melanocytic nevi with superimposed lichen sclerosus: A diagnostic challenge. J Am Acad Dermatol 2004; 50: 690-694.
La Spina M, Meli MC, De Pasquale R. Perrotta RE, et al. Vulvar melanoma associated with lichen sclerosus in a child: Case report and literature review. Pediatr Dermatol 2016; 33: e190-e194.
Notay M, Fazel N, Awasthi S. Cushing syndrome induced by topical corticosteroids for the treatment of lichen sclerosus. J Pediatr Adolesc Gynecol 2019; 32: 83-85.
Anderson K, Ascanio NM, Kinney MA, Krowchuk DP, Jorizzo JL. A retrospective analysis of pediatric patients with lichen sclerosus treated with a standard protocol of class I topical corticosteroid and topical calcineurin inhibitor. J Dermatol Treat 2016; 27: 64-66.
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