[Genital lichen sclerosus and lichen planus]. / Genitaler Lichen sclerosus und Lichen planus.

Lichen sclerosus (LS) and lichen planus (LP) are chronic inflammatory dermatoses of unknown aetiology. They pose the most important differential diagnoses of inflammatory dermatoses in the genital area. There is often a delay in diagnosing LS and LP and subsequently treatment is initiated late in the course of the disease, which will lead to scarring and a decreased quality of life. There is an increased risk of the development of malignancies in the genital area in both diseases; however, early and continuous treatment with potent topical steroids will decrease this risk.

Core outcome domains for lichen sclerosus: a CORALS initiative consensus statement.

BACKGROUND: Lichen sclerosus (LS) is a chronic inflammatory condition mainly affecting genital skin. It causes distressing symptoms that impact daily quality of life (QoL). It causes progressive anatomical changes and a potential risk of cancer. Published randomized controlled trials are of varying methodological quality and difficult to combine in meta-analyses. This is partly due to lack of agreed outcome measures to assess treatment response. Identification of core outcome sets (COSs), which standardize key outcomes to be measured in all future trials, is a solution to this problem. OBJECTIVES: To obtain international agreement on which outcome domains should be measured in interventional trials of genital LS. METHODS: Recommended best practice for COS domain development was followed: (i) identification of potential outcome domains: a long list was generated through an up-to-date LS literature search, including information collected during the LS priority-setting partnership; (ii) provisional agreement of outcome domains: a three-stage multi-stakeholder international electronic-Delphi (e-Delphi) consensus study; (iii) final agreement of outcome domains: online consensus meeting with international stakeholders including anonymized voting. RESULTS: In total, 123 participants (77 patients, 44 health professionals, 2 researchers) from 20 countries completed three rounds of the e-Delphi study. Eleven outcome domains were rated as 'critical' and were discussed at the online consensus meetings. The first set of consensus meetings involved 42 participants from 12 countries. Consensus was met for 'symptoms' (100% agreed) and 'QoL - LS-specific' (92% agreed). After the second set of meetings, involving 29 participants from 12 countries, 'clinical (visible) signs' also met consensus (97% agreed). CONCLUSIONS: The international community has agreed on three key outcome domains to measure in all future LS clinical trials. We recommend that trialists and systematic reviewers incorporate these domains into study protocols with immediate effect. CORALS will now work with stakeholders to select an outcome measurement instrument per prioritized core domain.

Lichen Sclerosus-Presentation, Diagnosis and Management.

BACKGROUND: Lichen sclerosus is a chronic inflammatory skin disease. It is thought to be underdiagnosed and undertreated. If it is not treated, lichen sclerosus is associated with a greater degree of scarring and an elevated risk of cancer in the genital area. METHODS: This review is based on pertinent articles published up to October 2015 that were retrieved by a selective search in PubMed, Embase, and the Cochrane Library and on the European S3 guideline for lichen sclerosus. RESULTS: Lichen sclerosus is mainly found in the anogenital area but can also be generalized. Extragenital involvement is reportedly present in 6% to 20% of patients. Neighboring mucous membranes, such as the vaginal or oral mucosa, are not typically affected. The disease is more common in women than in men, and occurs more often in adults than in children. About 10% of patients have other family members with the same condition. Anogenital lichen sclerosus often causes itching and pain. Functional impairment due to fissures and scars can arise over the course of the condition. The treatment of first choice is the local application of high-potency corticosteroids as early as possible (1/A). For boys and men in whom the condition does not remit after steroid treatment, circumcision is indicated (3/D). CONCLUSION: Anogenital itching and clinical features such as erythema, white skin changes (such as hyperkeratosis and sclerosis), and fissures should arouse suspicion of lichen sclerosus. The diagnosis should be confirmed with a skin biopsy, and early, thorough treatment should be initiated. In this way, a mutilating disease course can be averted, and the risk of cancer can be lessened.

Twelve-week treatment of lentigo maligna with imiquimod results in a high and sustained clearance rate.

Topical imiquimod cream is increasingly applied in the treatment of lentigo maligna (LM), in particular for large lesions where surgery may lead to disfiguring scars. Published studies suggest that more frequent and prolonged treatment with topical imiquimod is associated with higher efficacy. In this study we prospectively treated 27 patients suffering from LM on the face with imiquimod 5% cream using an intensive treatment regimen consisting of daily applications for 12 weeks inducing skin inflammation for at least 10 weeks. Twenty-four patients completed the treatment as recommended, 23 were available for follow-up (mean 39 months). Clinical and histo-pathological clearance was observed in 20 patients after a mean of 14 weeks of treatment. Notably, histopathological examination of a skin biopsy showed clearance of the LM in all 24 patients, including those who still showed some hyperpigmentation at 4 weeks off treatment. A clinical recurrence occurred in only 1 of the 23 patients available at follow-up. These findings suggest that the efficacy of imiquimod can be improved by implementing a more intensive treatment regimen. Randomized controlled trials are needed to confirm our results and establish the role of topical imiquimod in the treatment of LM.

Systematic review and meta-analysis of randomized controlled trials on topical interventions for genital lichen sclerosus.

BACKGROUND: Lichen sclerosus (LS) is a chronic inflammatory dermatosis that occurs mainly in the anogenital area and causes itching and soreness. Progressive destructive scarring may result in burying of the clitoris in females and phimosis in males. Affected people have an increased risk of genital cancers. OBJECTIVE: We sought to assess the effects of topical interventions for genital LS. METHODS: We undertook a systematic review and meta-analysis using the methodology of the Cochrane Collaboration. RESULTS: We included 7 randomized controlled trials with a total of 249 participants covering 6 treatments. Clobetasol propionate 0.05% was better than placebo in treating genital LS (participant-rated improvement/remission of symptoms: risk ratio 2.85 [95% confidence interval {CI} 1.45-5.61]; investigator-rated global degree of improvement: standardized mean difference [SMD] 5.74 [95% CI 4.26-7.23]) as was mometasone furoate 0.05% (change in clinical grade of phimosis: SMD -1.04 [95% CI -1.77 to -0.31]). We found no evidence supporting the efficacy of topical androgens and progesterone. There were no differences between pimecrolimus and clobetasol propionate in relieving symptoms through change in pruritus (SMD -0.33 [95% CI -0.99 to 0.33]) and burning/pain (SMD 0.03 [95% CI -0.62 to 0.69]). However, pimecrolimus was less effective than clobetasol propionate in improving gross appearance (investigator-rated global degree of improvement: SMD -1.64 [95% CI -2.40 to -0.87]). LIMITATIONS: Most of the included studies were small. CONCLUSIONS: The current limited evidence supports the efficacy of clobetasol propionate, mometasone furoate, and pimecrolimus in treating genital LS. Further randomized controlled trials are needed.

Topical interventions for genital lichen sclerosus.

BACKGROUND: Lichen sclerosus is a chronic, inflammatory skin condition that most commonly occurs in adult women, although it may also be seen in men and children. It primarily affects the genital area and around the anus, where it causes persistent itching and soreness. Scarring after inflammation may lead to severe damage by fusion of the vulval lips (labia); narrowing of the vaginal opening; and burying of the clitoris in women and girls, as well as tightening of the foreskin in men and boys, if treatments are not started early. Affected people have an increased risk of genital cancers. OBJECTIVES: To assess the effects of topical interventions for genital lichen sclerosus and adverse effects reported in included trials. SEARCH METHODS: We searched the following databases up to 16 September 2011: the Cochrane Skin Group Specialised Register, the Cochrane Central Register of Controlled Trials (CENTRAL) in The Cochrane Library, MEDLINE (from 2005), EMBASE (from 2007), LILACS (from 1982), CINAHL (from 1981), British Nursing Index and Archive (from 1985), Science Citation Index Expanded (from 1945), BIOSIS Previews (from 1926), Conference Papers Index (from 1982), and Conference Proceedings Citation Index - Science (from 1990). We also searched ongoing trial registries and scanned the bibliographies of included studies, published reviews, and papers that had cited the included studies. SELECTION CRITERIA: Randomised controlled trials (RCTs) of topical interventions in genital lichen sclerosus. DATA COLLECTION AND ANALYSIS: Two authors independently selected trials, extracted data, and assessed the risk of bias. A third author was available for resolving differences of opinion. MAIN RESULTS: We included 7 RCTs, with a total of 249 participants, covering 6 treatments. Six of these RCTs tested the efficacy of one active intervention against placebo or another active intervention, while the other trial tested three active interventions against placebo.When compared to placebo in one trial, clobetasol propionate 0.05% was effective in treating genital lichen sclerosus in relation to the following outcomes: 'participant-rated improvement or remission of symptoms' (risk ratio (RR) 2.85, 95% confidence interval (CI) 1.45 to 5.61) and 'investigator-rated global degree of improvement' (standardised mean difference (SMD) 5.74, 95% CI 4.26 to 7.23).When mometasone furoate 0.05% was compared to placebo in another trial, there was a significant improvement in the 'investigator-rated change in clinical grade of phimosis' (SMD -1.04, 95% CI -1.77 to -0.31).Both trials found no significant differences in reported adverse drug reactions between the corticosteroid and placebo groups. The data from four trials found no significant benefit for topical testosterone, dihydrotestosterone, and progesterone. When used as maintenance therapy after an initial treatment with topical clobetasol propionate in another trial, topical testosterone worsened the symptoms (P < 0.05), but the placebo did not.One trial found no differences between pimecrolimus and clobetasol propionate in relieving symptoms through change in pruritus (itching) (SMD -0.33, 95% CI -0.99 to 0.33) and burning/pain (SMD 0.03, 95% CI -0.62 to 0.69). However, pimecrolimus was less effective than clobetasol propionate with regard to the 'investigator-rated global degree of improvement' (SMD -1.64, 95% CI -2.40 to -0.87). This trial found no significant differences in reported adverse drug reactions between the pimecrolimus and placebo groups. AUTHORS' CONCLUSIONS: The current limited evidence demonstrates the efficacy of clobetasol propionate, mometasone furoate, and pimecrolimus in treating genital lichen sclerosus. Further RCTs are needed to determine the optimal potency and regimen of topical corticosteroids, examine other topical interventions, assess the duration of remission or prevention of flares, evaluate the reduction in the risk of genital squamous cell carcinoma or genital intraepithelial neoplasia, and examine the efficacy in improving the quality of the sex lives of people with this condition.

Treatment of lentigo maligna with imiquimod cream: a long-term follow-up study of 10 patients.

BACKGROUND: The first choice of treatment for lentigo maligna (LM) is excision. Initial studies of treatment with imiquimod 5% cream have shown promising results with excellent cosmetic outcome, but the follow-up duration in these studies was short. OBJECTIVES: To evaluate the results of treatment of patients with LM with imiquimod in routine clinical practice with long-term follow-up. METHODS: We prospectively followed 10 patients with LM who were treated with imiquimod 5% cream between 2004 and 2007 with a median follow-up of 31 months (range 11-56 months). Histological clearance was assessed in all patients using post-treatment biopsies. RESULTS: Complete clinical clearance was achieved in nine of 10 patients after treatment with imiquimod. During follow-up, three clinical and histological recurrences were observed at 9, 10, and 27 months after treatment cessation. In a fourth patient, histological recurrence without clinical signs was demonstrated 17 months after treatment. Five of 10 patients are in sustained clinical remission. CONCLUSIONS: Imiquimod appears to be an effective treatment for a subset of patients with LM. We recommend long-term follow-up and taking multiple post-treatment biopsies, even in the absence of a clinical recurrence. This case series emphasizes the need for finding an optimal treatment regimen.