Can vulvar edema be the presenting sign of Crohn's disease? A case series.

Vulvar involvement as an extraintestinal manifestation of Crohn's disease (CD) is a challenging diagnosis considering that vaginal findings may precede gastrointestinal symptoms. The aim of this study is to describe the clinical presentation, radiological findings, and treatment of vulvar Crohn's disease (VCD). We reviewed the time from initial presentation to diagnosis, presenting symptoms, radiological findings, gastrointestinal Crohn's disease Paris classification, and treatment response of five female pediatric patients. All the patients had radiological findings of vulvar inflammation on magnetic resonance imaging. Vaginal symptoms preceded gastrointestinal disease in two of the patients, which correlated with a delay in diagnosis. All patients had active disease on colonoscopy, with three of them having significant colorectal inflammation. Four of the patients were treated with infliximab, while one patient received ustekinumab with a resolution of their symptoms. In conclusion, VCD can precede gastrointestinal symptoms and is easily miss-diagnosed, leading to a delayed Crohn's disease diagnosis and treatment.

Effect of topical estrogen cream compared with observation in prepubertal girls with labial adhesions.

BACKGROUND: Topical estrogen treatment has been considered the first-line treatment of labial adhesions in prepubertal girls. However, the effect of topical estrogen cream is different according to studies, and no study compared estrogen cream to observation. OBJECTIVE: This study aims to investigate the efficacy of topical estrogen cream treatment compared with observation in prepubertal girls with labial adhesions. STUDY DESIGN: The medical records of prepubertal girls diagnosed with labial adhesions from April 2005 to June 2019 were retrospectively analyzed. Baseline characteristics such as age at diagnosis and initial symptoms were collected. The primary outcome was the resolution of labial adhesion. Secondary outcomes were recurrence and side effects. RESULTS: A total of 114 patients were enrolled and divided into two groups, topical estrogen cream (n = 94), and observation (n = 20). Girls who were treated with estrogen cream had older age (24.6 ± 19.0 vs. 16.7 ± 15.3 months, p = 0.037) and higher resolution rate than the observation group (100.0% vs. 85.0%, respectively, p = 0.005). Girls younger than 23.3 months showed a significantly higher resolution rate to topical estrogen treatment (100% vs. 86.7%, p = 0.043). Side effects and recurrences occurred exclusively in children treated with topical estrogen therapy without significant differences compared to the observation group. CONCLUSION: Topical estrogen therapy showed a higher resolution rate than observation for the treatment of prepubertal girls with labial adhesions, especially in younger girls.

Comparing quality of life in women with vulvovaginal lichen planus treated with topical and systemic treatments using the vulvar quality of life index.

BACKGROUND/OBJECTIVES: For patients with vulvovaginal lichen planus (VLP), there exists limited data on the comparison between patient quality of life treated with topical and/or systemic treatments. We characterised the treatment outcomes of VLP using the vulvar quality of life index (VQLI) comparing women treated with systemic immunosuppression, including humanised interleukin-23 monoclonal antibody tildrakizumab, to those treated with topical corticosteroids alone. METHODS: A retrospective cohort study is reported from a dermatology practice in Sydney, Australia. Electronic medical records for adult women with a diagnosis of VLP were reviewed identifying 112 subjects. VQLI scores in four domains (symptoms, activities of daily living, anxiety and sexual function) were compared between women able to maintain remission of disease with topical monotherapy to those with recalcitrant disease requiring treatment with conventional systemic immunosuppressants and for those not responding to this treatment, tildrakizumab. RESULTS: At baseline women requiring tildrakizumab treatment had the highest total VQLI score (24.6), whilst women whose disease was maintained on topical treatment had the lowest (19.2). Women treated whilst on tildrakizumab had significant reduced total mean VQLI scores (13.32, 95% CI 8.61-18.01) than when treated with other Systemic (22.00, 95% CI 16.52-27.53; p < 0.001) or topical (21.71, 95% CI 16.13-26.32; p < 0.01). Women treated with tildrakizumab demonstrated statistically significant decreases in mean VQLI scores in all four domains of the VQLI compared to previous scores when on other systemic treatments. CONCLUSION: We report the largest cohort study to date of adult women with VLP evaluating treatment responses to topical and systemic agents using the VQLI. In women whose VLP did not improve with conventional systemic immunosuppressants, tildrakizumab resulted in statistically significant decrease in mean VQLI scores in all 4 domains, highlighting tildrakizumab as an alternative treatment for VLP.

Lipschütz acute vulvar ulcer related to COVID-19 vaccination: First case report in South America.

Lipschütz ulcer is a non-sexually transmitted genital lesion of unknown etiology, which presents as a painful vulvar ulcer. Lipschütz ulcers have been described in most continents. This is the first case reported in Peru and South America. We present the case of a 33-year-old female patient with a Lipschütz ulcer after being vaccinated with the second dose of the AstraZeneca COVID-19 vaccine. She reported having had only one sexual partner in her lifetime. Laboratory results were negative for herpes simplex 2, Cytomegalovirus, Toxoplasma gondii, Epstein-Barr virus, and syphilis. The patient received symptomatic treatment. Ten days after the onset, the patient was significantly better during follow-up. This case report displays a potential adverse effect of the AstraZeneca COVID-19 vaccine as a Lipschütz ulcer triggered by the host humoral immune response. However, further research is needed to establish the causal relationship between these two.

La úlcera de Lipschütz es una lesión genital no transmitidas por vía sexual de etiología desconocida, se presenta como una úlcera vulvar muy dolorosa. Las úlceras de Lipschütz se han descrito en la mayoría de los continentes. Este es el primer caso que se reporta en Perú y Sudamérica. En este reporte de caso se presentauna paciente de 33 años con una úlcera de Lipschütz luego de haber sido vacunada con la segunda dosis de la vacuna AstraZeneca COVID-19. Refirió haber tenido una sola pareja sexual a lo largo de su vida. Los resultados de laboratorio fueron negativos para herpes simplex 2, Citomegalovirus, Toxoplasma gondii, virus de Epstein-Barr y sífilis. La paciente recibió tratamiento sintomático. Diez días después, durante el seguimiento, la paciente estaba significativamente mejor. Este reporte de caso expone un potencial efecto adverso de la vacuna AstraZeneca COVID-19, en forma de úlcera de Lipschütz, desencadenado por la respuesta inmune humoral del huésped. Sin embargo, es necesario realizar más investigación para establecer la relación causal entre ambos.

Vulvar ulcerative cutaneous tuberculosis secondary to genital tract tuberculosis.

Tuberculosis is a chronic infectious disease that gradually poses a certain threat to public health and economic growth. Tuberculosis typically affects the lungs, pleura, and lymph nodes and rarely the skin. Cutaneous tuberculosis manifesting as ulcerated lesions is also rare and often misdiagnosed and missed by clinicians. Here, the authors report a 29-year-old female patient presenting a vulvar ulcer for 10 months, accompanied by irregular menstruation and increased vaginal secretions. After a skin biopsy and endometrial PCR testing, it was finally diagnosed as vulvar ulcerative cutaneous tuberculosis secondary to genital tuberculosis. Anti-tuberculosis treatment was effective. Cutaneous tuberculosis is called a great imitator. In order to facilitate the diagnosis and treatment of tuberculosis by clinicians, the authors systematically reviewed this disease as well.

Vulvar dermatoses: a cross-sectional 5-year study. Experience in a specialized vulvar unit.

BACKGROUND: Vulvar diseases are common in the general population and have a negative impact on the quality of life. OBJECTIVES: To describe our experience as dermatologists in the management of vulvar dermatosis consultations. METHODS: A retrospective observational study was conducted with patients who attended monographic vulvar consultations over a 5-year period. Clinical information was obtained from the patient's charts. RESULTS: 148 women were studied. Their mean age was 43.24 years (standard deviation: 15.15 years), with ages ranging from 4 months to 80 years. 53.4% of patients took between 2 and 5 years to seek medical attention for the first time. The most frequent diagnosis was lichen sclerosus (41.9%), irritative eczema of the vulva (14.9%), and lichen simplex chronicus (10.1%). 83.8% reported anogenital itching, 66.2% pain, and 45.9% dyspareunia. The most frequently prescribed treatment was ultra-potent topical corticosteroids (clobetasol propionate; 41.2%). Patients with lichen sclerosus were significantly older than those who presented with any of the other diseases. No differences were found in terms of either the time of disease evolution or in symptom presentation. STUDY LIMITATIONS: Retrospective study. Vulvar diseases with an infectious cause are usually managed in primary care, therefore, were not included. All patients were recruited from a single private hospital which limits the comparisons with the public health system. CONCLUSIONS: Vulvar diseases frequently occur and are associated with high morbidity. It is essential to promote the development of specific vulvar consultations in hospitals. Specialties such as dermatology, gynecology, urology, or physiotherapy must be part of these units.

Quality of life impact and treatment response in vulval disease: Comparison of 3 common conditions using the Vulval Quality of Life Index.

BACKGROUND/OBJECTIVES: To compare the quality of life in patients with vulval lichen sclerosus (VLS), vulval lichen planus (VLP) and chronic vulvovaginal candidiasis (CVVC), as measured by the Vulval Quality of Life Index (VQLI). METHODS: A retrospective, single-centre cohort study was conducted at a combined dermatology and gynaecology practice from March 2018 to November 2021. VQLI scores and patient data were systematically collected and recorded in an online patient database. Treatment regimens were individualised and titrated to clinical response. RESULTS: Over 3 years, a total of 200 women were recruited: 59 with CVVC, 79 with VLP and 62 with VLS. The median duration of follow-up for all patients was 45.43 (16.25-80.89) weeks. At baseline, the median (interquartile range [IQR]) VQLI score was 24.00 (19.00-31.00), 21.00 (12.00-26.00) and 14.00 (7.00-26.00) for CVVC, VLP and VLS, respectively. At follow-up, the median (IQR) VQLI score for CVVC, VLP and VLS was 9.00 (3.00-15.00), 9.00 (3.00-16.00) and 5.00 (2.00-10.00), respectively. All three groups showed a significant improvement in VQLI score (p < 0.0001). At baseline, the highest scoring domains were 'Sexual Function' for CVVC and 'Future Health Concerns' for VLP and VLS. At follow-up, the highest scoring domains were 'Sexual Function' for CVVC and VLP, and 'Future Health Concerns' for VLS. CONCLUSIONS AND RELEVANCE: Vulval disease has an immense impact on QOL, especially in patients with CVVC. The VQLI is useful to clinicians in identifying the unique impact of each vulval condition on a patient's QOL in order to provide better patient-focussed care.

[PEDIATRIC AND ADOLESCENT GYNECOLOGY - VULVAR DISEASES].

INTRODUCTION: Vulvovaginitis and labial adhesion are the most common gynecological morbidity among girls and adolescents. Even though pediatricians or family physicians should be capable of dealing with these "ailments", in Israel, these patients are referred to gynecologists because physicians from these two medical specialties are deterred to treat these populations. Treatment is based on softening and antibiotic creams applied locally. In the current relevant literature the present notion regarding these two pathologies is shifted toward follow-up and habit changes in terms of proper hygiene and clothing. In the present review the relevant current literature is examined and updated recommendations are suggested.

Identifying predictors of systemic immunosuppressive treatment of vulvovaginal lichen planus: A retrospective cohort study of 122 women.

BACKGROUND: Between 20% and 40% of women with vulvovaginal lichen planus (VLP) fail to respond to first-line ultra-potent topical corticosteroid treatment and require systemic immunosuppression to control disease. No data exist regarding risk factors for patients with severe VLP requiring systemic immunosuppression. We aimed to identify clinical features that predict women with VLP who are at risk of severe, recalcitrant disease requiring eventual escalation to systemic treatment. METHODS: A chart review was performed on a cohort of 122 adult women with VLP followed prospectively for 15 years by the same clinician in a private dermatology practice in Australia between 1 January 2004 and 1 October 2021. Uni- and multivariable binary logistical regression analyses were performed to identify clinical features distinguishing women eventually requiring systemic treatment of VLP against those whose VLP was controlled with topical corticosteroids alone. RESULTS: The mean age at diagnosis of VLP was 61 years, with 35 women (28.7%) requiring systemic treatment of VLP. A multivariable regression model utilising 'Age at Diagnosis' (adjusted OR = 0.97, 95% CI 0.94-0.99), 'Non-Caucasian Ethnicity' (adjusted OR = 10.3, 95% CI 2.27-73.9) and 'Vulvar Pruritus' (adjusted OR = 2.69, 95% CI 1.11-6.86) demonstrated moderate predictive capacity, with specificity and sensitivity for predicting whether a patient will require systemic treatment for VLP of 95.3% and 40.5%, respectively. CONCLUSIONS: Our findings may indicate women with VLP who are younger, of non-Caucasian ethnicity, and presenting with vulval pruritus are the highest risk of severe disease requiring systemic immunosuppressive treatment and may benefit most from earlier initiation of systemic treatment.

Meet the expert: Topical management of vulvar dermatoses.

Vulvar dermatoses are often debilitating chronic skin conditions associated with pain and pruritus. In oncologic patients, cancer treatments can precipitate and exacerbate vulvar dermatoses. Cytotoxic chemotherapy, hormonal therapies, and local pelvic radiation therapy can lead to vulvar symptoms, and cancer treatment-induced vulvar conditions include graft-versus-host-disease and radiation dermatitis. There have also been reports of lichen sclerosus development or exacerbation secondary to hormonal therapy and immune checkpoint inhibitors, attributed to proposed hormonal and immunologic pathogenesis of lichen sclerosus. Early recognition and treatment of these conditions can significantly improve quality of life. In this review, we summarize the clinical features and management characteristics of six types of common vulvar dermatoses that may present in the oncologic patient.

Manual separation of labial synechiae: A cost-effective method in prepubertal girls.

BACKGROUND: Labial synechiae is a fairly common gynaecological problem that occurs as a result of inflammation leading to fusion between the labia minora. Being in a developing country with limited resources and poor compliance of patients to prolonged treatments, we are faced with immense challenges in the management of these girls. AIMS: We wanted to evaluate the efficacy and the cost-effectiveness of manual separation and topical antibiotics with perineal hygiene in the management of pre-pubertal girls with labial synechiae. MATERIALS AND METHODS: Design.Prospective, non-randomised interventional study. Participant: Pre-pubertal girls presenting with the diagnosis of labial synechiae in the period from September 2015 to January 2018. Interventions: Manual separation followed by topical antibiotic ointment application for 1 week with local hygiene. Outcomes measure: Complete release of the synechiae and no recurrence up to 6 months. RESULTS: Out of the total 55 patients, only 48 patients were included, their age ranged from 3 months to 7 years (mean 2.8 years). Almost half of our patients were asymptomatic, and other half had symptoms (urinary tract infection, dripping of urine and itching). Majority of our patients belong to low middle class status. We had 100% complete release of synechiae and no recurrence on 6 months follow-up. CONCLUSION: Manual separation followed by topical antibiotics is a cost-effective method of the treatment of labial synechiae with immediate response and low recurrence rate.

Vulvar Fordyce adenitis: A cohort of 45 women.

BACKGROUND: Recurrent episodes of painful papules and nodules, mostly located on the labia minora, have been reported under the denominations of vulvar sebaceous adenitis (14 published cases) and vulvar acne (16 published cases). OBJECTIVES: The primary aim of this study was to delve further into the clinical and pathological features of this condition. The secondary aim was to collect therapeutic data. METHODS: In this retrospective cohort study, files and photographs of patients with papules or nodules on the labia minora or the inner labia majora were extracted from a vulvar clinic database. Clinical, pathological and therapeutic data were analysed. RESULTS: Forty-five women were included from 2002 to 2018. The median age at the time of diagnosis was 36 years (range: 16-60). The median time to diagnosis was 6.5 years. Clinical features included recurrent painful papules, pustules or nodules, suppuration (n=22), and pitted scars (n=10) on the labia minora (n=41), the inner labia majora (n=19), the outer labia majora (n=1), and the clitoral hood (n=1). Associated acne vulgaris was seen in 17 out of 26 patients for whom data were available. Hidradenitis suppurativa, androgenic alopecia and hirsutism were observed in 3, 1 and 1 cases respectively. Neutrophilic infiltrates were observed in the 4 available biopsies within or around the Fordyce sebaceous glands in 2 patients. Treatment with tetracyclines and oral zinc was not consistently effective. Isotretinoin led to complete remission in 4 patients unresponsive to tetracyclines. DISCUSSION: Vulvar sebaceous adenitis/vulvar acne is a clinically identifiable cause of painful recurrent inflammatory lesions affecting the labia minora and the inner labia majora, which are coincidentally sites of Fordyce granules. We therefore suggest a more specific denomination: "vulvar Fordyce adenitis". The relationship with acne vulgaris and hidradenitis suppurativa requires further investigation. We suggest use of an oral anti-acne therapeutic strategy to reduce inflammation and to decrease the secretion of the sebaceous glands.

[Vulva bilharzia in a 10-year-old girl living in Kirane-Mali: a case report]. / Bilharziose vulvaire chez une fille de 10 ans à Kirane-Mali: rapport de cas.

Genital schistosomiasis is mainly located in the neck of the uterus and the vagina, less frequently on the vulva, the fallopian tubes and ovaries and rarely in the body of the uterus. We here report the case of a 10-year-old girl admitted with a swelling on the vulva in whom histological examination showed cutaneous schistosomiasis due to Schistosoma haematobium. Outcome was favorable with a single 40 mg/kg mg dose of praziquantel, with tumor regression.

Lipschütz Ulcer: An Unusual Diagnosis that Should Not be Neglected.

The diagnosis of genital ulcers remains a challenge in clinical practice. Lipschütz ulcer is a non-sexually transmitted rare and, probably, underdiagnosed condition, characterized by the sudden onset of vulvar edema along with painful necrotic ulcerations. Despite its unknown incidence, this seems to be an uncommon entity, with sparse cases reported in the literature. We report the case of an 11-year-old girl who presented at the emergency department with vulvar ulcers. She denied any sexual intercourse. The investigation excluded sexually transmitted infections, so, knowledge of different etiologies of non-venereal ulcers became essential. The differential diagnoses are extensive and include inflammatory processes, drug reactions, trauma, and malignant tumors. Lipschütz ulcer is a diagnosis of exclusion. With the presentation of this case report, the authors aim to describe the etiology, clinical course, and outcomes of this rare disease, to allow differential diagnosis of genital ulceration.

Labial adhesions in a pre-pubertal girl: The importance of awareness.

Labial adhesion affects up to 0.6-5% of pre-pubertal girls. They may be congenital or acquired. Patients usually are asymptomatic, and thus, labial adhesions are found incidentally on routine examination. If the patient is indeed truly asymptomatic, there is no need for any treatment, and reassurance only is needed. When treatment is indicated, topical application of oestrogen cream is advised, but when this fails, surgical intervention is recommended. Recurrence is common regardless of the treatment. We present the case of a three-year-old girl managed by release of adhesions under topical anaesthesia and topical application of oestrogen cream.

Lipschütz Ulcer: An Unusual Diagnosis that Should Not be Neglected

Abstract The diagnosis of genital ulcers remains a challenge in clinical practice. Lipschütz ulcer is a non-sexually transmitted rare and, probably, underdiagnosed condition, characterized by the sudden onset of vulvar edema along with painful necrotic ulcerations. Despite its unknown incidence, this seems to be an uncommon entity, with sparse cases reported in the literature. We report the case of an 11-year-old girl who presented at the emergency department with vulvar ulcers. She denied any sexual intercourse. The investigation excluded sexually transmitted infections, so, knowledge of different etiologies of non-venereal ulcers became essential. The differential diagnoses are extensive and include inflammatory processes, drug reactions, trauma, and malignant tumors. Lipschütz ulcer is a diagnosis of exclusion. With the presentation of this case report, the authors aim to describe the etiology, clinical course, and outcomes of this rare disease, to allow differential diagnosis of genital ulceration.