LICHEN SCLEROSUS
Treatment of vulvar lichen sclerosus with topical corticosteroids in children: a study of 72 children
Clin Exp Dermatol. 2014 Dec 5. doi: 10.1111/ced.12519. [Epub ahead of print]
Casey GA1, Cooper SM, Powell JJ.
Treatment of vulvar lichen sclerosus (VLS) in children by topical corticosteroids gives control of symptoms and some resolution of physical signs, but large studies are limited. We report the largest study of 72 prepubertal girls with VLS, 62 of whom were prospectively treated with daily application of an ultrapotent topical corticosteroid (UPTC), clobetasol propionate 0.05% ointment, for 3 months, with a follow-up period of 4-8 years [the remaining 10 patients responded to mild to moderate potency topical corticosteroids (MPTCs)]. The results were compared with a retrospective study of 31 prepubertal girls with VLS treated with MPTCs. MPTCs led to symptom clearance in 32.2% of patients, whereas UPTC led to symptom clearance in 72.6% of patients. Improvement in clinical signs following UPTC occurred in 90.3% of children at 3 months, with total resolution of clinical signs occurring in 29.2% at the 4-year follow-up or at puberty. No serious adverse effects occurred with UPTC treatment. In children with VLS, UPTCs relieve symptoms, resolve signs and possibly prevent scarring. UPTCs should therefore be the treatment of choice for VLS in children.
Lichen sclerosus in children and adolescents
Curr Opin Obstet Gynecol. 2013 Oct;25(5):370-4. doi: 10.1097/GCO.0b013e328363a53c.
Dendrinos ML1, Quint EH.
PURPOSE OF REVIEW: This review of lichen sclerosus in children and adolescents will discuss the disease and highlight the most recent literature.
RECENT FINDINGS: Lichen sclerosus continues to be poorly recognized and misdiagnosed by clinicians. There is growing support for an autoimmune component in the cause of this disease. Recent studies confirm that lichen sclerosus does not resolve after puberty but usually improves. In small case series, topical calcineurin inhibitors are effective as second-line therapy.
SUMMARY: Lichen sclerosus is an uncommon, poorly recognized disease in girls and adolescents and is likely to have a chronic course requiring long-term follow-up and treatment. There needs to be increased awareness among providers of this disease as a cause of vulvar itching. Because of the lack of knowledge of the natural course and treatment outcomes, prospective, multicenter studies are needed.
Clinical recommendation: pediatric lichen sclerosus
J Pediatr Adolesc Gynecol. 2014 Apr;27(2):111-6. doi: 10.1016/j.jpag.2013.11.004.
Bercaw-Pratt JL1, Boardman LA2, Simms-Cendan JS2;
Lichen sclerosus is a chronic inflammatory condition affecting the anogenital region that may present in the prepubertal or adolescent patient. Clinical presentations include significant pruritus, labial adhesions, and loss of pigmentation. Treatment includes topical anti-inflammatory agents and long-term follow-up as there is a high risk of recurrence and an increased risk of vulvar cancer in adult women with history of lichen sclerosus. These recommendations are intended for pediatricians, gynecologists, nurse practitioners and others who care for pediatric/adolescent girls in order to facilitate diagnosis and treatment.
Acute urinary retention in an adolescent as the presenting symptom of lichen sclerosus et atrophicus
J Pediatr Adolesc Gynecol. 2013 Dec;26(6):e117-9. doi: 10.1016/j.jpag.2013.03.006. Epub 2013 May 29.
Marcus-Braun N1, Hasan Z, Szvalb S, Ben Shachar I.
BACKGROUND: A rare case of acute urinary retention caused by labial fusion in an adolescent is described and the possible causes are discussed.
CASE: A 17-year-old girl, not sexually active, presented to our emergency service for acute urinary retention. Genital examination revealed labia minora fusion from the clitoris to the vaginal fourchette; urethra, and clitoris were not visualized. Sexual abuse and trauma were excluded. The labia minora were manually separated in the operating room revealing a normal vagina and urethral meatus. Skin biopsies taken from the fused labia minora revealed Lichen Sclerosus et Atrophicus.
SUMMARY AND CONCLUSION: Urinary retention may be seen in the face of complete adhesion of the labia minora, a rare event in postpubertal individuals. In such cases, a suspicion of underlying pathology such as asymptomatic Lichen Sclerosus should be raised and be confirmed by a biopsy.
Growing evidence for topical mometasone fuoroate in the treatment of Vulvar Lichen Sclerosus
Maturitas. 2014 Sep 27. pii: S0378-5122(14)00292-8. doi: 10.1016/j.maturitas.2014.09.005. [Epub ahead of print]
Virgili A1, Corazza M1, Minghetti S1, Borghi A2.
Vulvar lichen sclerosus (VLS) is a chronic inflammatory disease with a huge impact on a person's quality of life. On the basis of the immunologic changes in VLS, treatment with a high potency topical corticosteroid is recommended. The currently available guidelines identified the ultra-potent corticosteroid clobetasol propionate 0.05% (CP) as the first-line recommended treatment but growing evidence supports the use of the potent corticosteroid mometasone furoate 0.1% (MMF). We reviewed the available studies assessing MMF in the treatment of VLS. Both non comparative and comparative studies were included in this review. Findings from clinical trials indicate that MMF is an effective and safe option in the treatment of VLS both in the active phase and in long-term maintenance treatment. Consistent with the findings from the comparative studies, MMF shows similar efficacy and safety to CP.
Mometasone fuoroate 0.1% ointment in the treatment of vulvar lichen sclerosus: a study of efficacy and safety on a large cohort of patients
J Eur Acad Dermatol Venereol. 2014 Jul;28(7):943-8. doi: 10.1111/jdv.12219. Epub 2013 Jul 23.
Virgili A1, Borghi A, Minghetti S, Corazza M.
BACKGROUND: Guidelines identify a 3-month topical application of an ultra-potent corticosteroid ointment as the mainstay of medical treatment for vulvar lichen sclerosus (VLS). However, there are no trials providing evidence that any specific corticosteroid is superior to another.
OBJECTIVE: To assess the effectiveness and safety of a 12-week application of mometasone furoate (MMF) 0.1% ointment, with a tapering regimen, in achieving control of VLS signs and symptoms and to detect potential risk factors for VLS non-response.
METHODS: 147 patients affected with VLS were enrolled in a 12-week active treatment phase (ATP) with topical 0.1% MMF. The primary efficacy endpoint was the rate of patients achieving clinical response, as defined by protocol parameters. The secondary efficacy endpoint was to assess the changes of mean VLS-related symptoms after the 12-week ATP compared with baseline.
RESULTS: By the end of the ATP, 113 patients (80.7%) experienced a treatment response, whereas 27 women (19.3%) were judged as non-responders. Mean symptom scores decreased significantly in the study patients, regardless of their clinical response at the end of the ATP. Among all the epidemiological and clinical data considered, only the absence of previous medical therapies was found to be related to a significantly higher risk of non-response to treatment.
CONCLUSIONS: Application of 0.1% MMF ointment for 12 weeks on a tapering regimen was found to be an effective and safe therapy option in the ATP of VLS and could represent an alternative first-line treatment to an ultra-potent molecule.
Vulvar Lichen Sclerosus: A Comparison of the Short-term Topical Application of Clobetasol Dipropionate 0.05% Versus Mometasone Furoate 0.1%
J Low Genit Tract Dis. 2014 Jul 11. [Epub ahead of print]
Murina F1, Rehman S, Di Francesco S, Mantegazza V, Felice R, Bianco V.
OBJECTIVE: To compare the efficacy of the short-term application of clobetasol propionate 0.05% (CLB) and mometasone furoate 0.05% (MMF) in the treatment of vulvar lichen sclerosus (VLS).
STUDY DESIGN: This study was a retrospective review of 96 women who were diagnosed with lichen sclerosus with a follow-up of at least 1 year. Of these 96 women, 47 were treated with the topical application of CLB and 49 were treated with the topical application of MMF. All patients received treatment for 8 weeks. The drug was administered once daily for 4 weeks and then twice weekly for another 4 weeks in combination with a moisturizing cream that was continued as maintenance therapy. Subsequently, a topical steroid was applied on an as-needed basis if symptoms recurred. Follow-up visits were scheduled at 3, 6, and 12 months. The primary endpoint was the clinical response of the patients.
RESULTS: A mean of 77.3% of the patients were defined as responders at the 12-month observation, without any significant difference between the 2 treatment groups (CLB mean 73% vs MMF mean 81.6%, p = .2). A pronounced improvement in itching was observed after 3 months of treatment in the MMF group (p = .04), whereas the other symptoms were not significantly different between the 2 groups.
CONCLUSIONS: This study indicates that CLB 0.05% and MMF 0.1% in a short protocol regimen are effective, safe, and reliable treatments for VLS, with no significant difference between the 2 steroids in their efficacy. However, a moisturizing cream should be an integral part of the treatment.
First randomized trial on clobetasol propionate and mometasone furoate in the treatment of vulvar lichen sclerosus: results of efficacy and tolerability
Br J Dermatol. 2014 Aug;171(2):388-96. doi: 10.1111/bjd.12910. Epub 2014 Jul 23.
Virgili A1, Borghi A, Toni G, Minghetti S, Corazza M.
BACKGROUND: A 3-month topical application of clobetasol propionate (CP) represents the recommended and accepted first-line treatment for vulvar lichen sclerosus (VLS); however, to date, no randomized controlled trials have compared the efficacy and safety of CP with other topical corticosteroids.
OBJECTIVE: To compare the effectiveness and tolerability of two different topical corticosteroids, CP 0·05% ointment and mometasone furoate (MMF) 0·1% ointment, in the treatment of VLS.
METHODS: Fifty-four patients with VLS were enrolled in a 12-week active treatment phase (ATP) and randomized to apply either CP or MMF in a tapering regimen. The main efficacy parameters were the response rate, the rate of patients achieving an improvement from baseline of ≥ 75% in the subjective and objective scores, and the mean reduction in subjective and objective scores throughout the treatment.
RESULTS: By the end of the 12-week ATP, 24 (89%) patients were considered to be responders among the CP patients and 24 (89%) among the MMF patients; 59% and 37% of patients in the CP group and 67% and 48% in the MMF group achieved an improvement of at least 75% in subjective and objective scores, respectively. The decrease in mean symptom and sign scores was significant compared with baseline with both treatments. No significant differences were found in any of the assessed efficacy endpoints between CP and MMF. Both treatments were well tolerated.
CONCLUSIONS: Clobetasol propionate and MMF appeared similarly efficacious and well tolerated for the treatment of VLS and both may represent the first-line treatment for the disease.
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 Jul;71(1):84-91. doi: 10.1016/j.jaad.2014.02.019. Epub 2014 Apr 3.
Funaro D1, Lovett A2, Leroux N3, Powell J4.
BACKGROUND: Vulvar lichen sclerosus is a chronic condition usually responsive to topical corticosteroids.
OBJECTIVE: We sought to evaluate the efficacy (reduction of signs and symptoms) and safety of clobetasol propionate 0.05% and tacrolimus 0.1% in the treatment of vulvar lichen sclerosus.
METHOD: This double-blind, randomized study comparing 2 treatments over a 3-month period, enrolled 58 female patients with newly diagnosed vulvar lichen sclerosus or untreated vulvar lichen sclerosus for at least 1 month.
RESULTS: In all, 55 patients were included in the statistical analysis. A total of 28 patients were assigned to the tacrolimus group and 27 patients to the clobetasol group. Both groups showed a significant difference in the decrease of symptoms and signs of lichen sclerosus. At the end of the study, 28 participants (19 tacrolimus and 9 clobetasol) still had some clinical signs of lichen sclerosus (χ(2) = 6.56, P = .015). However, a significantly higher number of patients in the clobetasol group (n = 15) had absence of signs and symptoms of lichen sclerosus (χ(2) = 10.35, P = .002; χ(2) = 10.35, P = .002). No adverse events were reported.
LIMITATIONS: Short length of trial and recruitment through our vulvar disease referral center are limitations.
CONCLUSION: This study showed that topical clobetasol propionate was significantly more effective in treating vulvar lichen sclerosus than topical tacrolimus.
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(2):145-51. doi: 10.1159/000356163. Epub 2014 Feb 8.
Virgili A1, Borghi A, Toni G, Minghetti S, Corazza M.
BACKGROUND: Few reports have addressed the associations between clinical, demographic and historical variables of vulvar lichen sclerosus (VLS).
OBJECTIVE: To elaborate the prevalence and severity of signs and symptoms and to identify potential factors predicting the severity and course of VLS.
METHODS: A prospective cohort of 225 patients affected by VLS was included. Data were collected by direct interview and clinical examination.
RESULTS: 98% of patients complained of symptoms, principally itching. Pallor and scarring-sclerosis-atrophy were the most frequent and severe signs. The severity of VLS signs was not associated with age at onset and duration of the disease. About 70% of the patients had previously undergone treatment.
CONCLUSIONS: VLS-related symptoms were not associated with the clinical features which resulted less severe. Personal history of autoimmune diseases and familial history of VLS did not influence the age at onset and the severity of VLS. A considerable part of patients had previously received inappropriate treatment.
Hypertrophic lichen sclerosus sine sclerosis: clues to histopathologic diagnosis when presenting as psoriasiform lichenoid dermatitis
J Cutan Pathol. 2014 Nov 21. doi: 10.1111/cup.12457. [Epub ahead of print]
Weyers W1.
BACKGROUND: The histopathologic diagnosis of lichen sclerosus is usually facilitated by a subepidermal zone of sclerosis. In the absence of sclerosis, lichen sclerosus mostly presents itself as a psoriasiform lichenoid dermatitis that may be difficult to distinguish from other diseases.
OBJECTIVE: We sought to assess histopathologic findings that allow recognition of lichen sclerosus in the absence of sclerosis.
METHODS: We studied 28 criteria in 100 biopsy specimens of lichen sclerosus from genital or perianal skin, including 55 cases with marked sclerosis, 16 cases with mild sclerosis confined to foci of the papillary dermis, and 29 cases without sclerosis. 15 cases each of the early plaque stage of mycosis fungoides, lichen planus, and lichen simplex chronicus were studied for comparison.
RESULTS: Some histopathologic hallmarks of lichen sclerosus were seen chiefly in sclerotic lesions and, therefore, did not contribute to the diagnosis of difficult cases, such as dissolution of elastic fibers. Others were seen rarely in non-sclerotic lesions but might be helpful in individual cases, including follicular hyperkeratosis and thickening of the basement membrane. Findings that were more common and may be utilized as clues to the histopathologic diagnosis of non-sclerotic lichen sclerosus include tiny foci of homogenized tissue in dermal papillae, marked fibrosis with thickening of the papillary dermis, marked thickening of individual collagen fibers, lymphocytes aligned in rows between those fibers, necrotic keratinocytes, often with preserved pyknotic nuclei, in all reaches of the epidermis, including the cornified layer, clustering of necrotic keratinocytes above elongated dermal papillae, and vertical columns of parakeratosis with distinct dyskeratotic parakeratotic cells.