Clinical and pathological analysis of 345 cases of vulvar lichen sclerosus and a preliminary study on the frequency of maintenance treatment / 中华妇产科杂志

Objective: To analyze and summarize the clinical and pathological characteristics, management, and efficacy of patients with vulvar lichen sclerosus (VLS) through a single center large sample study, and preliminarily to explore the frequency of maintenance treatment medication for VLS. Methods: The clinical data of VLS patients in Obstetrics and Gynecology Hospital of Fudan University from 2018 to 2021 were retrospectively collected. The clinicopathological characteristics (patients' age, course of disease, complicated disease history, family history, symptoms, signs and pathology), treatment and effects were retrospectively analyzed. The patients in the maintenance treatment stage were followed up regularly to explore the minimum frequency of individual medication to maintain the stability of the disease. Results: (1) General situation: a total of 345 patients with VLS were included in this study. The average age was (50.4±14.7) years (ranged from 8 to 84 years old), prevalence was highest in the 50-59 years group (30.1%, 104/345). Immune diseases occurred in 18.6% (33/177) of patients, 24.3% (43/177) of patients had allergic skin diseases, and 5.6% (10/177) of the patients' immediate family members had chronic vulvar pruritus or vulvar hypopigmentation. (2) Clinical features: the most common symptom was vulvar pruritus (96.1%, 196/204) among 204 patients with recorded symptoms. The most common sign was hypopigmentation of the vulva (96.3%, 206/214). The most common involved sites were labia minora (70.3%, 142/202), labia majora (67.8%, 137/202), and labial sulcus (59.4%, 120/202). The cumulative number of sites involved in 62 vulvar atrophy patients (2.7±1.1) was significantly higher than that in 152 non-atrophy patients (2.2±1.0; t=3.48, P=0.001). The course of vulvar atrophy was (9.3±8.5) years, which was significantly longer than that of non-atrophy patients [(6.6±5.6) years; t=2.04, P=0.046]. (3) Pathological features: among the 286 patients with electronic pathological sections, the most common pathological feature in the epidermis was epithelial nail process passivation (71.3%, 204/286). The common pathological features in the dermis were interstitial collagenization (84.6%, 242/286), and inflammatory cell infiltration (73.8%, 211/286). (4) Treatment: 177 patients received standardized treatment after diagnosis and were followed up regularly in our hospital. In the initial treatment stage, 26.0% (46/177) of the patients were treated with 0.05% clobetasol propionate cream, and 74.0% (131/177) of the patients were treated with 0.1% mometasone furoate ointment. The complete remission rates of the two methods were respectively 80.4% (37/46) and 74.0% (97/131), and there was no statistically significant difference (χ²=0.76, P=0.385). During maintenance treatment, 27.1% (48/177) of the patients took the medication twice a week, 35.0% (62/177) took the medication once a week, and 37.9% (67/177) took the medication once every 10 days. During follow-up after 6 months of maintenance treatment, there were no patients with recurrence of pruritus or progression of vulvar signs. Conclusions: The majority of VLS patients have itching, hypopigmentation, involvement of labia minora and labia majora, progressive atrophy, and inflammatory infiltration of dermis. Local treatments of mometasone furoate and clobetasol propionate have good initial therapeutic effects. The frequency exploration of individualized maintenance treatment could minimize the occurrence of adverse reactions when ensuring the stability of the patients' condition.

Microablative fractional radiofrequency as a therapeutic option for vulvar lichen sclerosus: a pilot study

OBJECTIVES: To assess the clinical response to and the histomorphometric effects of microablative fractional radiofrequency (MFR) in women with symptomatic vulvar lichen sclerosus (VLS). METHODS: This was a pilot study on the use of MFR for the treatment of VLS. Upon recruitment and at each treatment session, all participants were examined and each of their symptoms were rated on a visual analog scale. After the procedure, the participants completed a satisfaction questionnaire. We compared the morphometric findings of vulvar biopsies performed at enrollment and after the last treatment session. The participants were divided into three groups according to previous treatment with corticosteroids: G1, no previous treatment; G2, treated for up to 5 years; and G3, treated for >5 years. RESULTS: This study included 26 women. After two to three sessions, most participants in all groups became either "asymptomatic" or "much better" than before treatment and were "very satisfied" or "satisfied" with the intervention. Pruritus and burning sensation were the most frequently reported symptoms. Nearly 40% of the participants in all groups reported complete remission of symptoms. The improvement was rated as moderate or higher by 80%, 76%, and 66% of the women in groups 1, 2, and 3, respectively. The improvement of symptoms persisted for 11 months (range, 7-16 months), on average, after the treatment. Type III collagen concentration significantly increased and was associated with important symptom improvement. Tissue trophism and vascularization also increased but did not reach statistical significance, probably because of the small number of cases. CONCLUSIONS: MFR may be an effective and safe treatment for symptomatic VLS.

A radiofrequência fracionada no tratamento de líquen escleroso vulvar: um relato de caso / Fractional radiofrequency in the treatment of vulvar lichen sclerosus: a case report

O líquen escleroso vulvar (LEV) é uma doença dermatológica crônica de etiologia incerta, caracterizada por prurido intenso e atrofia progressiva. O corticosteroide tópico de longo prazo é o tratamento de primeira linha para LEV. No entanto, esse tratamento requer a colaboração da paciente, está associado a efeitos colaterais adversos e algumas pacientes não respondem aos corticosteroides. O tratamento com tecnologias térmicas e fototérmicas tem sido estudado como terapia alternativa ou complementar para melhorar os sintomas de LEV e o trofismo cutâneo. A radiofrequência fracionada microablativa é usada em dermatologia para melhorar o trofismo tecidual. Também tem sido usada em pacientes ginecológicas para tratar a atrofia vulvovaginal, estimulando a neocolagênese dérmica e a neoelastogênese. Apresentamos o caso de uma mulher de 39 anos com LEV refratária que foi tratada com aplicações locais de radiofrequência fracionada microablativa. Ela apresentou melhora satisfatória dos sintomas e do trofismo vulvar em longo prazo, sem necessidade do uso de corticosteroides.(AU)

Vulvar lichen sclerosus (VLS) is a chronic dermatological disease of unclear etiology characterized by severe itching and progressive atrophy. Long-term topical corticosteroid is the first-line treatment for VLS. However, this treatment requires patient compliance, is associated with adverse side effects, and some patients do not respond to corticosteroids. Treatment with thermal and photothermal technologies have been studied as alternative or complementary therapies to improve VLS symptoms and skin trophism. Microablative fractional radiofrequency (MFR) is used in dermatology to improve tissue trophism. It has also been used in gynecological patients to treat vulvovaginal atrophy by stimulating dermal neocollagenesis and neoelastinogenesis. We present the case of a 39-year-old woman with refractory VLS who was treated with local applications of microablative fractional radiofrequency. She had satisfactory, long-term, improvement of symptoms and vulvar trophism, and stopped using corticosteroids.(AU)

Rejuvenation Using Platelet-rich Plasma and Lipofilling for Vaginal Atrophy and Lichen Sclerosus

Vaginal atrophy is a common condition among peri- and post-menopausal women. Symptoms of vaginal dryness, pruritus, irritation, loss of subcutaneous fat, sparse pubic hair and dyspareunia occur due to decreased estrogen level. Estrogen-based treatments are effective. But many patients are reluctant to be treated due to health concerns. As alternatives, we explored the efficacy of platelet-rich plasma (PRP) and lipofilling. A 67-year-old female patient with vaginal atrophy was referred to our department. Treatment using estrogen cream had failed to improve patient's symptoms. Diminished volume and aged look of genitalia were also major concerns. We treated her using lipofilling mixed with PRP. A total of 40 cc of autologous fat mixed with PRP was transferred to labia majora. Lipofilling with PRP relieved the clinical symptoms. Missing fullness and tone was corrected and the augmented volume was well maintained. White patchy lesions of lichen sclerosus on labia minora also improved. Lipofilling with PRP relieved symptoms, restored contour of the labia majora and achieved remission of lichen sclerosus on labia minora. As vulvar lesions were repaired and the aged appearance of genitalia was rejuvenated, both functional and cosmetic outcomes were satisfactory. Lipofilling with PRP can be effective for vaginal atrophy and lichen sclerosus.

The Role of Nanofat Grafting in Vulvar Lichen Sclerosus: A Preliminary Report

No abstract available.

Conscious sedation with inhaled 50% nitrous oxide/oxygen premix in photodynamic therapy sessions for vulvar lichen sclerosus treatment

Photodynamic therapy has been described as an effective therapeutic option in selected cases of anogenital lichen sclerosus that are refractory to first-line treatments. However, procedure-related pain is a limiting factor in patient adherence to treatment. The authors report the case of a 75-year-old woman with highly symptomatic vulvar lichen sclerosus, successfully treated with photodynamic therapy. An inhaled 50% nitrous oxide/oxygen premix was administered during sessions, producing a pain-relieving, anxiolytic, and sedative effect without loss of consciousness. This ready-to-use gas mixture may be a well-tolerated and accepted alternative to classical anesthetics in Photodynamic therapy, facilitating patients' adherence to illumination of pain-prone areas.

Tratamiento de liquen escleroso familiar con tacrolimus tópico al 0.1% / Treatment of family lichen sclerosus with tacrolimus 0.1% ointment

El liquen escleroso es una enfermedad inflamatoria mucocutánea crónica, de etiología desconocida, que afecta principalmente las áreas anogenitales.Habitualmente se presenta con placas blancas escleróticas, acompañadas de sequedad de la mucosa, prurito intenso y persistente, que conduce a la atrofia progresiva e impotencia funcional. Presentamos dos hermanas con liquen escleroso vulvar de difícil manejo, que respondieron con éxito al tratamiento con tacrolimus ungüento al 0,1%...

Liquen escleroso vulvar en la infancia. Reporte de un caso en una niña con síndrome de Turner / Vulvar lichen sclerosus in a girl with Turner syndrome

Las afecciones dermatológicas en el síndrome de Turner son poco frecuentes, pero en algunos casos ocasionan morbilidad significativa. El liquen escleroso vulvar (LE) es una afección inflamatoria mucocutánea crónica, caracterizada por prurito en la zona anogenital. Su fisiopatogenia se vincula con factores genéticos y autoinmunes. Se presenta el caso de una niña con síndrome de Turner en tratamiento con hormona de crecimiento, que consultó por prurito vulvar, cuyo diagnóstico correspondió a liquen escleroso.

Dermatological complications in Turner syndrome are infrequent but occasionally cause significant morbidity. Lichen sclerosus (LS) is a chronic inflammatory mucocutaneous affection characterized by pruritus in the anogenital area. It is yet not clear its pathophysiology but it's linked with genetic factors and autoimmunity. This is a case report of a girl with Turner syndrome with growth hormone treatment that started with vulvar pruritus and was diagnosed as lichen sclerosus

Os três líquens: escleroso, plano e plano erosivo / The three lichens: sclerosus, planus and erosive

Líquen escleroso (LE) e líquen plano (LP) são doenças de natureza inflamatória que atacam a pele e a região anogenital e que, por vezes, podem ser de difícil diferenciação clínica em seus respectivos estágios iniciais. O LE teve sua incidência demonstrada em 1,7% dos atendimentos ginecológicos, em todas as faixas etárias. Já o LP ataca cerca de 1% das mulheres, mas sua incidência real é difícil de avaliar. LE e LP são doenças cuja patogênese é multifatorial. Fatores genéticos e hormonais e infecções e trauma foram associados ao desenvolvimento de LE, enquanto que LP tem seu desenvolvimento menos compreendido; no entanto, também é associado a fatores genéticos, a infecções e ao uso de certos medicamentos. As duas doenças causam bastante desconforto às pacientes, pois podem se apresentar com dor, ardência, prurido e também há a possibilidade de estenose do introito vaginal e consequente dispareunia. Na suspeita de LE e LP, deve ser feito diagnóstico diferencial com outras doenças de pele e com neoplasia intraepitelial vulvar (NIV). O risco de transformação maligna justifica o acompanhamento que as pacientes devem ter pelo resto da vida. O tratamento de primeira linha para LE e LP consiste em corticosteroides tópicos superpotentes. O diagnóstico requer frequente biópsia para sua confirmação. O objetivo deste trabalho foi revisar a fisiopatologia, complicações e tratamento dessas duas doenças, as quais são muito importantes, pois não possuem cura e podem comprometer seriamente a qualidade de vida das pacientes.(AU)

Lichen sclerosus (LS) and lichen planus (LP) are diseases of an inflammatory nature that attack the skin and anogenital region, and sometimes they might have a difficult clinical differentiation in their early stages. LE has its incidence demonstrated in 1.7% of gynecological consultations in all age groups. LP attacks about 1% of women, but its true incidence is difficult to assess. LE and LP are diseases whose pathogenesis is multifactorial. Genetic and hormonal factors, infections and trauma were associated with the development of LE, while LP has its development less understood; however, it is also associated with genetic factors, infections and the use of certain medications. Both diseases cause much discomfort to the patient, because they may present with pain, burning, itching and there is also the possibility of stenosis of the vaginal opening and consequent dyspareunia. On suspicion of LE and LP should be done differential diagnosis with other skin diseases and vaginal intraepithelial neoplasia (VIN). The risk of malignant transformation justifies the monitoring that patients should have in the rest of their life. The first-line treatment for LE and LP consists of topical corticosteroids super powerful. The diagnosis often requires biopsy for confirmation. The aim of this study was to review the pathophysiology, complications and treatment of these diseases, which are very important because they have no cure and can seriously compromise the quality of life of patients.(AU)

The Co-Existence of Vulvar Lichen Sclerosus, Ulcerated Calcinosis Cutis, and Dermatomyositis: Coincidence or Immunological Mechanism?

Calcinosis cutis is a condition characterized by the deposition of calcium salts in the skin and subcutaneous tissues, and patients suffering from it encounter various connective tissue disorders, such as dermatomyositis (DM), scleroderma, and systemic lupus erythematosus. Although calcinosis cutis is frequently accompanied by juvenile dermatomyositis, rare cases have been reported in adult patients with DM. On the other hand, lichen sclerosus (LS) is a chronic inflammatory disease of the skin and mucosal surfaces. In the present report, we present a rare case of a 71-year-old patient with DM accompanied by ulcerated calcinosis cutis and vulvar LS.

Trasplante capilar en una paciente con alopecia fibrosante frontal asociada con liquen escleroatrófico de vulva / Hair transplantation in a patient with frontal fibrosing alopecia associated with lichen scleroatrophicus vulvae

La alopecia fibrosante frontal (AFF) puede representar una variedad del liquen plano pilaris. Se presenta una paciente con AFF asociada con liquen escleroatrófico de vulva que fue tratada con finasteride (1 mg/día) y aplicaciones tópicas de minoxidil a 2% dos veces al día. Se le efectuaron microtrasplantes de pelo en la zona alopécica frontal y frontoparietal, con resultado excelente que se mantiene 6 años después. Los microtrasplantes capilares pueden ser considerados una alternativa terapéutica no convencional.

Liquen escleroso vulvar y carcinoma espinocelular / Vulvar lichen sclerosus and squamous cell carcinoma

Introducción. El liquen escleroso es una entidad que se caracteriza por afectar piel y mucosas. Por su localización de elección a nivel vulvar, es abordado tanto por ginecólogos como por dermatólogos. Se debe realizar un seguimiento exhaustivo de estas pacientes con la finalidad de mejorar la calidad de vida y evitar su transformación a carcinoma espinocelular. Objetivo. Determinar la incidencia de liquen escleroso en nuestro medio y su relación con el carcinoma espinocelular vulvar.Material y métodos. Se incluyeron en el estudio 32 pacientes con diagnóstico de liquen escleroso genital evaluadas en el Consultorio de Patología Vulvar en un período de cinco años. Resultados. El liquen escleroso representó el 7,05% de las consultas en el Consultorio de Patología Vulvar. De las 32 pacientes evaluadas, el rango de edad fue de 50 a 82 años, con una media de 66 años.La incidencia familiar fue observada en un caso (3,12%), en el que se vieron afectadas madre e hija. En el 96% de las enfermas, el síntoma preponderante fue el prurito.De las 32 pacientes con liquen escleroso, 6 (18,75%) se asociaron a carcinoma espinocelular; de éstos, 4 (66,7%) correspondieron a VIN diferenciado y 2 (33,3%) a carcinomas invasores. Durante el seguimiento, una de las pacientes falleció a causa del tumor. Conclusiones. El liquen escleroso es una patología frecuente que debería ser evaluada en forma conjunta por dermatólogos y ginecólogos. La transformación maligna en el 18,75% de nuestros casos justifica un seguimiento exhaustivo tanto clínico como histopatológico con la finalidad de detectar en forma temprana la aparición de neoplasias.

Liquen plano erosivo vulvar asociado a granuloma de sílice: reporte de un caso / Vulvar erosive lichen planus associated with silica granulom: case report

Se presenta el caso de una paciente mujer de 52 años con prurito intenso y dolor en vulva con tres años de evolución, quien usó varios tratamientos tópicos como corticoides y talco para calmar el prurito. El estudio histopatológico reveló dos patrones de infiltrado, el primero, un infiltrado liquenoide con atrofia de la epidermis compatible con liquen plano erosivo vulvar, y el segundo, un infiltrado profundo y organizado en granulomas sin necrosis gaseosa, con múltiples células gigantes a cuerpo extraño y con un material cristalino en su interior que a la microscopía de luz polarizada se reveló como espículas brillantes birrefringentes compatibles con silice. Se presenta el caso por ser la primera descripción de asociación entre liquen plano erosivo vulvar y granuloma a sílice.

We present the case of 52 year old women who had a three year history of severe itching and pain in vulva, for which she used several topical treatments such as corticosteroids and tale to soothe the itching. The histopathology study revealed two patterns of infiltration, the first, a lichenoid infiltrate with atrophy of the epidermis consistent with vulvar erosive lichen planus, and the second, a deep infiltrate organized in granulomas without caseous necrosis, with multiple foreign body giant cells and a crystalline material inside, which in polarized light microscopy revealed itself as bright birefringent spicules, compatible with silica. We present this caseas the first description of an association between vulvar erosive lichen planus and silica granuloma.

O papel do gene p16 e do gene Dapk no controle do ciclo celular e na via carcinogênese vulvar / The role of p16 and Dark genes in cell cycle control and in the pathways of vulvar carcinogenesis

O câncer vulvar é o quarto tipo de câncer mais comum nas mulheres e representa 4,8% dos cânceres do trato genital inferior. O carcinoma de células escamosas é responsável por 80 a 90% de todos os cânceres de vulva. O carcinoma escamoso vulvar e suas lesões pré-malignas parecem desenvolver-se por dois caminhos distintos, baseados em características etiológicas e histopatológicas, tendo assim uma etiologia heterogênea. Um dos caminhos está relacionado com a infecção pelo HPV, e o outro, com as desordens epiteliais, tais como líquen escleroso e hiperplasia epitelial. O HPV é um importante fator causal das neoplasias do trato genital inferior. Ele está presente em cerca de 90% dos cânceres do colo uterino e 30 a 40% dos cânceres de vulva. O tipo mais prevalente é o 16, seguido pelos tipos 18, 45, 31 e 33. O estudo das alterações genéticas e epigenéticas, por meio da análise de metilação e imunoexpressão gênica, tem demonstrado uma grande versatilidade para o monitoramento molecular de pacientes com câncer, o que impulsiona pesquisas de métodos diagnósticos e terapêuticos do câncer. Nesta atualização pretendeu-se demonstrar as funções dos genes p16 e DAPK e as recentes pesquisas sobre a expressão destes genes nas vias da carcinogênse vulvar.

Vulvar cancer is the fourth commonest kind of cancer in women and it represents 4.8% of cancers in the lower genital tract squamous cell carcinoma is responsible for 80-90% of all vulvar cancers. Squamous cell carcinoma and it's premalignant lesions seem to develop in two distinct pathways, based on etiological and histopathological characteristics, thus forming a heterogeneous etiology. Whereas one of the pathways is related to HPV infection, the other is related to epithelial disorders such as: lichen sclerousus and epithelial hyperplasia. HPV is an important contributing factor of neoplasia in the lower genital tract. It is found in 90% of cervical cancers and in 30-40 % of vulvar cancers. The most prevalent kind is 16, followed by 18, 45, 31, and 33. The study of genetic and epigenetic alterations by means of methylation and genic immunoexpression has demonstrated great versatility to the monitoring ofpatients with cancer, which boosts researches of diagnostic and therapeutic methods for cancer. This update intends to demonstrate the role of p16 and DAPK genes as well as the recent researches regarding the expression of these genes in the pathways of vulvar carcinogenesis.

Study on the risk factors of 100 cases with vulvar dystrophy / 中华流行病学杂志

<p><b>OBJECTIVE</b>To assess the risk factors for vulvar dystrophy.</p><p><b>METHODS</b>An epidemiological study was carried out. Data on 100 cases with vulvar dystrophy was reviewed and face to face interviewed with a uniform questionnaire including the manner of work, environmental temperature, habit of eating, mood, underwear wearing, autoimmune diseases, marriage, menstrual age, the quantity of menses, orders of pregnancy, and labor trauma of vulvar during delivery, vulvitis and urethritis ect. Univariable analysis and multivariate logistic regression analysis were carried out with 1:1 case-control methodology.</p><p><b>RESULTS</b>Multiple conditional logistic regression analysis showed that vulvar dystrophy was positively associated with hot food (OR = 2.55, 95% CI: 1.24 - 5.25), mood (OR = 4.27, 95% CI: 1.96 - 9.29), order of pregnancy (OR = 3.37, 95% CI: 2.11 - 5.40), vulvitis (OR = 6.74, 95% CI: 2.66 - 17.09) and urethritis (OR = 11.02, 95% CI: 1.01 - 120.19). Vulviitis or urethritis increased 6.74 or 11.02 times the incidence of vulva dystrophy. Anger or nervous state contributed to the incidence of vulva dystrophy (OR = 4.27). Addict to hot food and order of labor also increased risk ratio for 2.55 and 3.37 times, respectively.</p><p><b>CONCLUSION</b>The risk factors of vulvar dystrophy were: addict to hot food, often holding a angry or nervous state, increase of labors, having vulvitis and urethritis.</p>

Lichen sclerosus et atrophicus [Hallopeau] and related conditions historical, nosologic and clicico-pathologic studies

Six cases of Lichen Sclerosus et Atrophicus were studied. Genital affection was detected in 50% of the cases. Of most significance is the finding that the histopathologic picture corresponds with the stage of the pathologic process, being variable in different lesions at different phases and at different sites of the same lesion