Nd:YAG/Er:YAG dual laser vs. topical steroid to treat vulvar lichen sclerosus: study protocol of a randomized controlled trial.

PURPOSE: Vulvar lichen sclerosus (LS) is a chronic debilitating inflammatory skin disease. Today, the gold standard is a life-long topical steroid treatment. Alternative options are highly desired. We present a study protocol of a prospective, randomized, active-controlled, investigator-initiated clinical trial comparing a novel non-invasive dual Nd:YAG/Er:YAG laser therapy with the gold standard for the management of LS. METHODS: We recruited 66 patients, 44 in the laser arm and 22 in the steroid arm. Patients with a physician-administered clinical LS score ≥ 4 were included. Participants received either four laser treatments 1-2 months apart, or 6 months of topical steroid application. Follow-ups were planned at 6, 12, and 24 months. The primary outcome looks at the efficacy of the laser treatment at the 6-month follow-up. Secondary outcomes look at comparisons between baseline and follow-ups within the laser or the steroid arm, and comparisons between laser vs. steroid arm. Objective (LS score, histopathology, photo documentation) and subjective (Vulvovaginal Symptoms Questionnaire, symptom VAS score, patient satisfaction) measurements, tolerability, and adverse events are evaluated. CONCLUSION: The findings of this trial have the potential to offer a novel treatment option for LS. The standardized Nd:YAG/Er:YAG laser settings and the treatment regime are presented in this paper. CLINICAL TRIAL IDENTIFICATION NUMBER: NCT03926299.

Morphea patients with mucocutaneous involvement: A cross-sectional study from the Morphea in Adults and Children (MAC) cohort.

BACKGROUND: Demographic and clinical findings of patients with mucocutaneous morphea have not been well characterized, to our knowledge. OBJECTIVE: To determine the demographic and clinical characteristics of morphea patients with mucocutaneous lesions who were enrolled in the Morphea in Adults and Children cohort. METHODS: Cross-sectional study of 735 patients in the Morphea in Adults and Children cohort from 2007 to 2018. RESULTS: A total of 4.6% of linear morphea patients had oral involvement versus 2.4% among the entire cohort, whereas 10.3% of generalized morphea patients had genital involvement versus 3.7% among the entire cohort. Patients with genital lesions were older at disease onset than those with oral morphea (57 versus 11.5 years; P < .001) and had more frequent extragenital lichen sclerosus et atrophicus (59.2% versus 5.6%; P = .004). LIMITATIONS: Selection bias and limited number of affected subjects. CONCLUSION: Oral morphea lesions predominate in younger patients with facial linear morphea, whereas genital lesions predominate in postmenopausal women with overlying extragenital lichen sclerosus et atrophicus.

Identification and management of vulval problems of the postmenopausal woman - Tips and tricks.

Vulval irritation and discomfort can be a common presentation to both primary and secondary care. These symptoms can become increasingly prevalent due to physiological changes, which occur to the female genitalia following menopausal transition or due to inflammatory conditions. The correct diagnosis and management can have a huge impact on the patients' quality of life. However, due to the nature of the symptoms, there can be delayed presentation to healthcare professionals. This article gives an overview of the most common benign vulval conditions in the post-menopausal woman, their clinical features and the diagnosis and initial management.

The Vulval Disease Quality of Life Index in women with vulval lichen sclerosus correlates with clinician and symptom scores.

BACKGROUND/OBJECTIVES: The Vulval disease Quality of Life Index (VQLI) is a new tool that assesses the burden of vulval disease on quality of life (QoL). Our objective was to assess the correlation between VQLI score and clinician-rated severity scores, overall patient itch/discomfort, disease duration, sexual activity, and age, in vulval lichen sclerosus (VLS) at a vulval disorders clinic. METHODS: A retrospective case note review, including consecutive women with VLS who attended the clinic between April and October 2018. Outcome measures include the VQLI score, clinician-rated severity score, and patient symptom score. RESULTS: A total of 109 women with VLS were included. On multivariable analysis, there was evidence of a positive relationship between VQLI scores and the total clinician-rated score (mean increase in VQLI score per unit increase in clinician score 1.34, 95% confidence interval [CI] 0.31, 2.38; P = 0.01); the relationship was stronger for the cutaneous component. There was little evidence for relationships of the VQLI with the patient's age, sexual activity or time since onset of symptoms. There was strong evidence for a positive relationship between VQLI score and overall itch/discomfort score (mean increase 2.38, 95% CI 1.88, 2.88; P < 0.001). New and follow-up data were obtained on sequential visits for 12 women, among whom the VQLI score dropped a mean -2.75 points between visits (95% CI -6.05, 0.55; P = 0.094). CONCLUSION: The clinician-rated severity correlates with the impact of VLS on QoL. The VQLI captures information included in a patient itch/discomfort score, which can be easily incorporated into routine assessment.

Paediatric vulval lichen sclerosus: a retrospective study.

BACKGROUND: Lichen sclerosus (LS) is a chronic inflammatory dermatosis with a predilection for the anogenital region, which mainly affects prepubertal girls and postmenopausal women. The cause is unknown, but a number of potential aetiological factors have been identified. AIM: To examine a cohort of patients with prepubertal-onset vulval LS (VLS) and assess baseline characteristics, clinical presentation, potential precipitating and predisposing factors, and response to treatment. METHODS: Data were collected from case notes on patients aged < 18 years diagnosed with prepubertal-onset VLS attending a specialist vulval dermatology service. Data included clinical presentation, comorbidities, family history, therapy and response to treatment. RESULTS: In total, 26 paediatric patients were identified. The median age at onset of symptoms was 5 years (range 2-8.5 years). Many previously identified potential aetiological factors for the development of VLS 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. CONCLUSION: A detailed assessment is essential in patients with VLS so that potential predisposing factors and comorbidities can be identified and managed. Urinary incontinence may be implicated in the development of paediatric VLS and may prevent adequate disease control. Paediatric VLS can persist through puberty, thus long-term follow-up is advised.

Level of use and safety of botanical products for itching vulvar dermatoses. Are patch tests useful?

BACKGROUND: Topical remedies based on botanical ingredients are popular. OBJECTIVES: To assess: (i) the usage of botanical substances in subjects affected with itching and chronic vulvar complaints; (ii) the incidence of side-effects associated with their use and the frequency of contact allergy; (iii) the diagnostic usefulness of patch testing. METHODS: Sixty-six patients were provided with a questionnaire to assess the prevalence and type of topical botanical preparations used and the occurrence of adverse reactions. Patients were patch tested with (i) the Italian baseline series, (ii) a topical medicament series, and (iii) a botanical series. RESULTS: Forty-two patients (63.6%) reported the use of natural topical products on the vulva. Seven (16.7%) noted adverse reactions; 27 showed positive reactions with the baseline series; 14 (21.2%) had at least one relevant reaction, mainly to allergens in topical products and cosmetics; and 2 (3%) showed positive reactions to the botanical series. Of the 7 patients complaining of adverse effects of botanical products, 3 (42.8%) showed relevant sensitization. CONCLUSIONS: The use of natural topical products is widespread among women affected with itching vulvar diseases. Contact dermatitis is a possible adverse effect. Botanical series are of questionable usefulness, owing to the wide variety of botanical ingredients.

What differentiates symptomatic from asymptomatic women with lichen sclerosus?

AIMS: To investigate the differences between symptomatic and asymptomatic women with vulvar lichen sclerosus (LS). METHODS: Data on file concerning 228 LS patients was retrospectively reviewed. RESULTS: Most patients were symptomatic (193/228; 85%). Worsening of symptoms related to specific food intake was experienced by 26% (48/182) and pork was the most frequently cited (25/48; 52%); worsening at night was reported by 47% (87/185). Being over 50 years at the time of diagnosis or being menopausal were associated with being symptomatic (RR = 2.47, 95% CI: 1.15-5.29 and 6.55, 2.05-20.91, respectively). The same was true for urinary incontinence (UI) (5.23, 1.78-15.42), the use of absorbents (without UI) (3.59, 1.05-12.34) and dyspareunia (5.28, 1.64-17.0). All patients with a family history of LS were symptomatic. There was no association with hormonal factors or specific vulvar anatomical changes. CONCLUSIONS: Pork consumption may be involved in the worsening of symptoms. UI and the use of absorbents are more commonly present in symptomatic LS patients. Dietary changes and correction of UI might be useful in the control of LS symptoms. © 2015 S. Karger AG, Basel.

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

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

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.

Photodynamic therapy of vulvar lichen sclerosus et atrophicus in a woman with hypothyreosis--case report.

Lichen sclerosus et atrophicus (LSA) is disease of skin and mucosa, its pathogenesis remains unknown. Itching, pain and burning sensations and atrophy of vulva impair quality of life. Treatment is symptomatic. We report case of 30-year old woman with lesions in vulva in which series of topical PDT were carried out. We applied Levulan®Kerastick® for 4h and after that lesions were illuminated with red light. Along with above treatment patient started receiving Euthyrox®, because of recently diagnosed hypothyreosis. Significant relief from subjective symptoms was achieved and lesions in vulvar region disappeared. Combination of topical PDT with hormonal therapy allowed controlling course of disease and minimizing symptoms, and thus improved quality of life.

A population-based case-control study of aetiological factors associated with vulval lichen sclerosus.

We aimed to investigate the association between possible aetiological factors and the risk of developing vulval lichen sclerosus (VLS). A population-based case-control questionnaire study was performed comparing women with a diagnosis of VLS (n= 92), with those attending a general gynaecology clinic with no known anogenital dermatosis (n= 66). After adjustment for confounders, factors associated with VLS included a family history of diabetes mellitus (OR= 7.0, p= 0.012) and previous pelvic surgery (OR= 4.75, p= 0.007). The use of barrier and progesterone only methods of contraception (OR= 0.19, p= 0.045), hormone replacement therapy (OR= 0.209, p= 0.025) or hayfever (OR= 0.18, p= 0.008) appeared to be associated with a reduced risk of VLS. In conclusion, we were unable to confirm many proposed aetiological theories associated with the development of VLS, in particular those associated with autoimmunity.

Erosive diseases of the vulva.

Vesicobullous and inflammatory diseases produce vulvar erosions that may exhibit nonspecific morphology and represent a diagnostic challenge. An approach to arriving at the correct diagnosis is presented. Most common etiologies are reviewed.

Chlamydia trachomatis infection in women with lichen sclerosus vulvae and vulvar cancer.

OBJECTIVE: Chronic infections in the urogenital area often precede or coexist with vulvar cancer. A strong connection between some tumours and the-appearance of Chlamydia trachomatis infection has been observed, but there is little information concerning a connection of that infection with vulvar cancer and lichen sclerosus vulvae (LS). The aim of this study was the analysis of frequency of antigens appearance and antibodies of IgM and IgG Chlamydia trachomatis in patients with vulvar cancer and LS and we wanted to find the correlation between Chlamydia trachomatis infection and vulvar cancer and LS. METHODS: 80 women treated in the Clinic of Vulva Diseases at the Department and Clinical Ward of Gynaecology, Obstetrics and Oncological Gynaecology in Bytom, in the Silesian Medical University in Katowice were divided into two groups - 30 were treated for vulvar cancer and 50 were treated because of LS. We took bacterial smears vagina and cervical smears for presence of Chlamydia trachomatis antigens and peripheral blood to mark antibodies of IgM and IgG Chlamydia trachomastis. RESULTS: Chlamydia trachomatis antigen was found in 20% women with vulvar cancer and in 12% women with LS (p>0.05). In 13,3% cases with vulvar cancer we observed IgM Chlamydia trachomatis antibodies. In the group with LS IgM antibodies appeared in 16% women (p>0.05). In 50% patients with vulvar cancer in blood serum we observed IgG Chlamydia trachomatis antibodies, and in 16% women with LS (p<0.001). CONCLUSIONS: Previous Chlamydia trachomatis infection can lead to vulvar carcinogenesis.

Paediatric vulval lichen sclerosus.

Lichen sclerosus (LS) in children is uncommon; however, it is an important differential diagnosis in any pre-pubertal child presenting with chronic vulval symptoms. The long-term prognosis is unknown; however, recent data suggests that the assumption that the condition will resolve at puberty may be incorrect. Children with LS require long-term management with topical corticosteroids, which remains the treatment of choice, as well as long-term follow up. We review the current literature on paediatric vulval LS.

Hypoxia-ischaemia is involved in the pathogenesis of vulvar lichen sclerosus.

BACKGROUND: Lichen sclerosus (LS) is a chronic inflammatory skin disease, the pathogenesis of which is poorly understood. AIM: To evaluate the role of hypoxia-ischaemia (HI) in vulvar LS. METHODS: Samples from five patients with vulvar LS and five control subjects were collected for analysis by transmission electron microscopy (TEM) to reveal the ultrastructural changes of organelles and dermal blood capillaries. Samples from 37 patients with vulvar LS and 12 control subjects were collected for immunohistochemistry to detect the expression of vascular endothelial growth factor (VEGF) and the hypoxia markers hypoxia-inducible factor (HIF)-1alpha and glucose transporter (Glut)-1. RESULTS: Using TEM, the mitochondria of basal cells and vascular endothelial cells in vulvar LS tissue were found to be swollen with loss of cristae, and the rough endoplasmic reticulum had luminal swelling and ribosomal detachment. Damage to vascular endothelial cells, disorganization of capillary architecture and loss of capillaries were also seen. By immunohistochemistry, moderate to intense staining of VEGF was seen in almost 90% of control sections vs. about 55% of LS sections. Glut-1 expression was negative or weak in 75% of control sections vs. moderate to very strong in about 80% of vulvar LS sections. Nuclear staining of HIF-1alpha was not found in LS or control tissue. CONCLUSIONS: HI is involved in the pathogenesis of vulvar LS.

Review of squamous premalignant vulvar lesions.

Vulvar squamous cell carcinoma (SCC) develops following two different pathways, which have their own premalignant lesions. In the absence of human papilloma virus (HPV), vulvar SCC can develop in a background of lichen sclerosus (LS), differentiated vulvar intraepithelial neoplasia (VIN) or both. The other pathway leading to vulvar SCC is associated with HPV and the HPV-associated premalignancy is usual VIN. In this review we will discuss the history, epidemiology, aetiology, histology, clinical characteristics, treatment options, malignant potential and prevention strategies of the three squamous premalignant vulvar lesions.

Use of acellular human dermal allograft for vaginoplasty in Mayer-Rokitansky-Küster-Hauser syndrome: a case report.

BACKGROUND: Both autologous and synthetic tissue have been used to create a neovagina in women with Mayer-Rokitansky-Küster-Hauser syndrome. Despite reports on many different techniques, the ideal method of vaginoplasty has not been firmly established. CASE: A 33-year-old woman with Mayer-Rokitansky-Küster-Hauser syndrome had been unsuccessful in using vaginal dilators for the creation of a functional vagina with the Frank technique due to pain and vulvar lichen sclerosis. She thus elected to undergo vaginoplasty by a modified McIndoe procedure using acellular human dermal allograft material. Both the surgery and immediate postoperative course were uncomplicated. Vaginal apex stenosis resulted from inadequate dilatation in the weeks following surgery since the patient experienced pain with dilator use. CONCLUSION: The use of dermal allograft material instead of a split-thickness autologous skin graft is a simple and less invasive method of constructing a neovagina. Due to the many potential advantages of this technique, further investigation is warranted to optimize long-term outcomes of vaginoplasty using this method.