Vulvar skin disease in the pediatric population.

PURPOSE OF REVIEW: Vulvar skin disease is an underrecognized pediatric condition encompassing a wide range of conditions, from isolated vulvar disease to vulvar manifestations of systemic illnesses. This review highlights the most current research discussing clinical features, risk factors, and treatments. RECENT FINDINGS: Recent studies confirm that labial adhesions resolve more quickly with estrogen treatment. Topical corticosteroids remain first-line for treatment of vulvar lichen sclerosus, and some procedural interventions are showing promising results. Latest evidence shows efficacy of biologic agents in hidradenitis suppurativa and vulvar Crohn's. Vaginal voiding remains an underrecognized cause of irritant vulvovaginitis. Lately vulvar aphthae have been associated with coronavirus disease 2019. Distinguishing between infantile perianal pyramidal protrusion, molluscum, and condyloma can be aided by differences in morphologic features. SUMMARY: Vulvar dermatoses have a high impact on children's health and wellbeing. Clinician familiarity with recognition and latest advancements in vulvar dermatoses can aid in prompt diagnosis, management, and appropriate referrals. Vulvar biopsy and vaginal cultures should be used prudently. Treatments include topicals, behavioral modification, systemic medications, and procedures.

Comparison of Word Catheter and Marsupialization in the Management of Bartholin's Glands: A Systematic Review and Meta-Analysis.

OBJECTIVE: There is no consensus on the best option for the management of Bartholin's gland abscesses. This systematic review and meta-analysis aimed to compare the Word catheter placement and marsupialization methods in the management of women with a cyst or abscess of the Bartholin's glands. DATA SOURCES: The PubMed, Scopus, Embase, Web of Science, and Cochrane Library databases, as well as Google Scholar, were searched to retrieve articles published between January 1990 and July 2023, comparing the Word catheter treatment with marsupialization for women with a cyst or abscess of the Bartholin's glands. STUDY SELECTION: Both observational studies and randomized controlled trials (RCTs) were included in this meta-analysis. DATA EXTRACTION AND SYNTHESIS: After the review, out of 9 relevant articles, only 4 (2 observational studies and 2 RCTs) were included in this meta-analysis, providing the data of 735 patients (396 in the Word catheter group vs. 339 in the marsupialization group). The data was extracted from the selected articles, using a data extraction form. Comparison of the Word catheter and marsupialization methods suggested that the risk of recurrence was approximately 7.6% in the Word catheter group and 9.4% in the marsupialization group. The findings indicated no significant difference in the recurrence of cysts or abscesses when comparing the Word catheter treatment with marsupialization (odds ratio = 0.99, 95% confidence interval: 0.29-3.43, P = 0.990, I2 = 77%). CONCLUSION: This meta-analysis found no significant difference in the rate of recurrence between the Word catheter and marsupialization methods. In general, marsupialization and Word catheter placement in the treatment of Bartholinitis seem to be equally effective.

Insights from a joint pediatric dermatology-gynecology vulvar clinic: A retrospective study.

BACKGROUND/OBJECTIVES: Pediatric vulvar disease has not been widely explored in the medical literature. Few studies focus on vulvar disease in skin of color. The vulvar disease can be distressing for young patients given the sensitive location, and providers may lack experience in diagnosing and managing vulvar dermatoses. We sought to characterize the conditions seen, diagnostic challenges encountered, and the racial and ethnic factors associated with vulvar diseases in our multidisciplinary pediatric dermatology-gynecology vulvar clinic at Children's National. METHODS: Medical records of 220 patients who presented to our multidisciplinary pediatric dermatology-gynecology clinic were reviewed retrospectively. RESULTS: Lichen sclerosus (LS) (36%, n = 80), inflammatory vulvitis (11%, n = 23), and vitiligo (9%, n = 19) were the three most frequent conditions observed. These conditions were often misdiagnosed as one another. There was a mean delay in diagnosis after symptom onset in LS patients of 16.43 months. CONCLUSIONS: LS, inflammatory vulvitis, and vitiligo are common vulvar diseases among pediatric patients. Accurate diagnosis is important because LS must be treated aggressively to prevent sequelae. Further studies are warranted to help differentiate LS and vitiligo with consideration of skin tone.

Beyond vulvodynia: from a correct diagnosis to a multidisciplinary care program. A referral center experience.

BACKGROUND: Vulvodynia is a chronic pain condition without an identifiable cause. As such, it is a diagnosis of exclusion, and all other causes of vulvar pain should be excluded. Although a standard treatment for vulvodynia has not been established yet, multidisciplinary care programs appear to be effective. PUROPOSE: The aim of this retrospective monocentric study was to analyze the prevalence of vulvodynia among women referred to our institution for a suspected diagnosis and to evaluate the efficacy of a multidimensional treatment plan. The primary outcome was the prevalence of vulvodynia following differential diagnosis. Secondary outcomes included: prevalence of the differential diagnoses, symptom resolution rate following treatment, and the relation between persistence of symptoms and (a) patients' age; (b) coexisting chronic overlapping pain conditions (COPCs). RESULTS: After having ruled out all other causes of vulvar pain, only 40.1% of women were considered as affected by vulvodynia. The most frequent differential diagnoses included lower genital tract infections (25.3%), vulvar lichen sclerosus (17.6%) and vulvovaginal atrophy (8.2%). Following a multidisciplinary care program, resolution of symptoms was observed in 13.6% cases, improvement in 64.3% and persistence in 21.9%. We did not find a statistically significant association between persistence of symptoms and age > 38 years (OR 2.10; p = 0.30). Women with one or more COPCs other than vulvodynia had a 75% increased risk of not obtaining a resolution of symptoms (OR 1.75; p = 0.44). CONCLUSION: A thorough differential diagnosis and a multidisciplinary care program may represent a first way out of the muddle in the management of these patients.

Vulvar Ulcers: An Algorithm to Assist With Diagnosis and Treatment.

OBJECTIVES: Vulvar ulcers can be challenging to diagnose, manage, and treat. Ulcers can be nonspecific in appearance and have many etiologies. Description of the lesion is very important. METHODS: An interactive vulvar ulcer algorithm was created to aid in the evaluation, diagnosis, and treatment of vulvar ulcers. RESULTS: The algorithm flowchart begins with careful history and physical examination. Pending these, specific tests can be obtained to aid in diagnosis. The algorithm also links to appropriate treatments. The algorithm can be accessed on the International Society for the Study of Vulvovaginal Disease Web site ( issvd.org ). Each underlined word in the algorithm is a hyperlink that leads to a wealth of information on the topic that providers can use to direct testing and aid in diagnosis and treatment. CONCLUSIONS: The vulvar ulcer algorithm can help clinicians with diagnosis and treatment plans.

Early treatment of vulvar synechiae in childhood - prevention of late complications.

Synechiae of the vulva occurs especially in girls in the so-called resting hormonal period with a minimal serum concentration of estrogens. If the external genitalia of the girl are not treated properly, the mucous membrane of the vaginal entrance is not regularly checked and mechanically removed, then the smegma of the opposite mucous membranes of the vulva can stick together. The place of the adhesion of epithelium in the vaginal introitus can be covered by a new epithelium and can create a firm adhesion, synechiae of the vulva, which is usually asymptomatic and is discovered incidentally during pediatric examination. Clinically significant vulvar synechiae can cause impaired bladder evacuation and recurrent lower urinary tract infections. Dissection of synechiae in the stage of conglutination or thin adhesion is performed manually on an outpatient basis, but extensive and firm adhesions must be divided surgically under general anesthesia. Local application of cream with estriol or betamethasone is also used for the therapy of synechiae of the vulva, but this treatment is more time-consuming compared to rapid manual separation and requires good compliance of the girls and their parents. The prevention of synechiae is based on the education of parents by pediatricians and gynecologists about the need for regular checks of the external genitalia and sufficient hygiene.

The efficacy and safety of secondary focused ultrasound therapy for recurrence of non-neoplastic epithelial disorders of the vulva.

OBJECTIVE: To investigate the efficacy, safety, and influencing factors of secondary focused ultrasound (FU) therapy for recurrence of non-neoplastic epithelial disorders of the vulva (NNEDV). METHODS: Patients with NNEDV who have relapsed after initial FU treatment were included in this study. They were treated with secondary FU therapy between July 2015 and July 2021. Outcome measures included the degree of symptom severity and operative complications. We further analyzed the relationships between age, course, time between relapse and initial treatment, menopause status, lesion size, pathological types, severity of symptoms, and curative rate. RESULTS: There were 98 patients enrolled in this study, with a mean age of 47.4 ± 11.4 years. All patients successfully underwent secondary FU therapy. Blisters developed among 16 (16.3%) patients, of whom 6 (6.1%) developed superficial skin ulcers. A curative response was observed among 46 (46.9%) patients, while an effective response was observed among 44 (44.9%) patients. Only 8 (8.2%) patients showed no improvement. The total response rate was 91.8%. A total of 12 (12.2%) cases recurred among all effective cases. Patients with a recurrence of NNEDV after more than 1.5 years following their first FU therapy demonstrated a higher response rate than those with a recurrence after less than 1.5 years. CONCLUSIONS: A second FU therapy remains effective for patients with recurrent NNEDV with no obvious side effects. The response rate, however, is higher for patients who experience recurrence of NNDEDV after more than 1.5 years.

[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.

Vulvar Crohn's Disease: Clinical Features and Outcomes.

INTRODUCTION: Vulvar involvement is a rare complication of Crohn's disease (CD). The optimal treatment of vulvar CD is unknown. METHODS: We conducted a 25-year retrospective cohort study of vulvar CD from 3 referral centers. Clinical features and outcomes were studied. RESULTS: Fifty patients were identified. The most common vulvar symptoms were pain (74%), edema (60%), ulcerations (46%), nodules (36%), and abscess (34%). Medical management leading to symptomatic improvement varied, and 5 patients ultimately required surgery. DISCUSSION: Vulvar CD manifests with a broad spectrum of symptoms. Aggressive medical management was frequently effective, although surgery was required in 10% of cases.

Vulvovaginal manifestations in Stevens-Johnson syndrome and toxic epidermal necrolysis: Prevention and treatment.

The prevalence of acute vulvovaginal involvement in toxic epidermal necrolysis can be as high as 70%; up to 28% of female patients will also develop chronic vulvovaginal sequelae. There is little consensus regarding prevention and treatment of the gynecologic sequelae of both Stevens-Johnson syndrome and toxic epidermal necrolysis (SJS/TEN). We review acute and chronic sequelae, including erosions, scar formation, chronic skin changes, urethral complications, adenosis, malignant transformation, vulvodynia, and dyspareunia. We provide comprehensive recommendations for acute and long-term vulvovaginal care in adult and pediatric SJS/TEN patients. Treatment should include an ultrapotent topical steroid, followed by a nonirritating barrier cream applied to vulvar and perineal lesions. A steroid should be used intravaginally along with vaginal dilation in all adults (but should be avoided in prepubertal adolescents) with vaginal involvement. Menstrual suppression should be considered in all reproductive age patients until vulvovaginal lesions have healed. Last, referrals for pelvic floor physical therapy and to surgical subspecialties should be offered on a case-by-case basis. This guide summarizes the current available literature combined with expert opinion of both dermatologists and gynecologists who treat a high volume of SJS/TEN patients.

Factors influencing the clinical efficacy of high-intensity focused ultrasound in the treatment of non-neoplastic epithelial disorders of the vulva: a retrospective observational study.

BACKGROUND: High-intensity focused ultrasound (HIFU) is an important method for treating non-neoplastic epithelial disorders of the vulva (NNEDV), but the factors affecting the efficacy of HIFU for NNEDV treatment remain unclear. OBJECTIVE: To determine the factors influencing the clinical efficacy of HIFU in the treatment of NNEDV. METHODS: A retrospective observational study of 186 NNEDV patients treated with HIFU was performed to evaluate the therapeutic effect, and the relationship between treatment efficacy and clinicopathological factors was analyzed. RESULTS: NNEDV signs and symptoms were effectively improved and relieved after HIFU treatment. The total effective and recurrent rates were 95.16% and 4.52%, respectively. Younger patients with shorter disease courses, mild itching, or peri-vulvar lesions had better therapeutic effects, and patients with anxiety and/or depression had worse treatment outcomes. CONCLUSION: The therapeutic effect of HIFU on NNEDV is not only related to the itching degree, age, or disease course but is also closely related to lesion location as well as anxiety and depression in patients.

Management of postmenopausal vulvovaginal atrophy: recommendations of the International Society for the Study of Vulvovaginal Disease.

OBJECTIVE: To develop a best practice document for the management of postmenopausal vulvovaginal atrophy (VVA). METHOD: Literature review carried out using clinical terms, treatments or interventions and comorbidity related to VVA. RESULTS: There is a wide variety of interventions that may produce temporal benefits for VVA. However, there are significant limitations in scientific publications concerning VVA and related issues, including variable outcome evaluations, variability in population age range, and small, often underpowered sample sizes. Therapeutic management of VVA should follow a sequential order, considering women's age, symptoms, general health as well as treatment preference. Beneficial options include lubricants, moisturizers, vaginal estrogens (estradiol, estriol, promestriene, conjugated estrogens), androgens, prasterone, and laser application. In women with general menopausal symptoms who are candidates for systemic hormone therapy, the lowest effective dose should be used. Oral ospemifene is an effective selective estrogen receptor modulator to treat VVA. Systemic androgens have a limited role. Although laser procedures are commonly used, at this moment the International Society for the Study of Vulvovaginal Disease does not endorse its use out of the setting of clinical trials. Pelvic floor muscle training improves blood flow and elasticity of the vulvovaginal tissue. In breast cancer survivors, moisturizers and lubricants are first line therapy. However, limited absorption of low/ultra-low doses of estrogens suggests safety, especially in women under treatment with aromatase inhibitors. As clinical practice and available preparations vary between countries this text should be adapted to local circumstances. CONCLUSIONS: There is a wide range of therapeutic options to individualize VVA treatments.

Anogenital giant condyloma in an infant with liver transplantation.

Human papillomavirus (HPV) types 6 and 11 were detected in a 3-year-old girl with extensive anogenital condylomata. Although sexual abuse must be considered, non-sexual transmission is evident in at least 57% of children with anogenital warts. Perinatal transmission may occur in approximately 24.5% of infants born to HPV-positive mothers. We present an immunosuppressed child with giant condylomata and discuss transmission, work up, and treatment.

Vulvar diseases: Conditions in adults and children.

The most problematic vulvovaginal conditions are familiar to dermatologists but may exhibit distinct clinical features or medication management because of the anatomic location. The second article in this continuing medical education series focuses on management pearls for treating vulvar diseases. We highlight key conditions, such as lichen sclerosus, erosive lichen planus, and vulvodynia. In addition, we review conditions that dermatologists may be less familiar with, such as plasma cell vulvitis, desquamative inflammatory vaginitis, vulvar aphthae, and low estrogen states. Nearly 1 in 6 women experience undiagnosed and untreated vulvovaginal discomfort at some point in their lives. Physicians who treat vulvar disorders will improve the quality of life of countless women.

Effectiveness of hyaluronate-based pessaries in the treatment of vulvovaginal atrophy in postmenopausal women.

Objectives: This study aimed to assess the efficacy and safety of hyaluronic acid-based vaginal pessaries (Hydeal-D) in the treatment of vulvovaginal atrophy (VVA).Study design: The study was a prospective, multicenter clinical investigation of VVA topical treatment in 40 postmenopausal women. Patients applied one Hydeal-D pessary every 3 days for 3 months.Main outcome measures: The primary endpoint was the amelioration of VVA signs after treatment, evaluated by measuring the change from baseline of the Vaginal Health Index (VHI) score. Secondary endpoints included the evaluation of other VVA-related signs and symptoms, safety, and patient-reported and clinician-reported satisfaction and treatment tolerability.Results: The 3-month treatment with Hydeal-D vaginal pessaries showed efficacy for all analyzed endpoints. Improvement exceeded threshold values of VVA diagnosis, sexual dysfunction, and distress, confirming clinically relevant amelioration of VVA symptoms. Changes from baseline conditions confirmed significant improvement of all parameters including the VHI, vaginal pH, patients' perception of VVA symptoms, sexual function, and vaginal maturation. Patients' overall satisfaction was very high after 1 month of treatment and increased further after 3 months. No severe adverse events were reported.Conclusions: Significant amelioration of VVA-related signs indicates that Hydeal-D vaginal pessaries are an effective, safe, and well-tolerated non-hormonal therapeutic option for VVA in postmenopausal women.

Ospemifene plus fractional CO2 laser: a powerful strategy to treat postmenopausal vulvar pain.

This study is a single-center, retrospective analysis of postmenopausal women presenting with dyspareunia and vulvar pain, aiming to evaluate relative effectiveness of vestibular CO2 laser therapy as a treatment. Three monthly sessions of laser were performed to each patient and thereafter a three-months follow-up was stablished. A total number of 72 patients undergoing vestibular laser treatment were recruited from patient files in the period between 2016 and 2018. Among these, 39 women also received a concomitant treatment with ospemifene (60 mg/day) during the study period. There was a statistically significant reduction of all the symptoms in both groups up to the three month follow-up. Regarding dryness and dyspareunia, the relief tent to be more prominent in the ospemifene + laser group at all follow-ups and remained statistically significant at three-month follow-up. Specifically, vestibular dryness was significantly lower in the ospemifene + laser group compared with the laser treatment group (-87% vs - 34%, respectively), and the vestibular health score started declining faster in the ospemifene + laser group. Although, additional research is needed to understand the mechanism of action, our data shows that a combination regimen of laser and ospemifene may improve clinical effectiveness for long-term treatment of symptoms associated with the under-recognized genitourinary syndrome of menopause.

Review of non-invasive vulvovaginal rejuvenation.

Vulvovaginal atrophy (VVA) or genitourinary syndrome of menopause (GSM) may affect up to 90% of menopausal women. Features include vulvovaginal atrophy, vulvovaginal laxity, vulvovaginal dryness and irritation, dyspareunia, anorgasmia and urinary symptoms. The vulva, vagina and bladder are oestrogen-responsive tissues, but oestrogen replacement therapy is not possible in women who have hormone-responsive cancers or normal oestrogen levels. Therefore, there is a role for alternative treatments. To date, three non-surgical energy-based therapies have been proposed: fractional microablative CO2 laser, erbium:YAG laser and temperature-controlled radiofrequency (RF). Our objective was to assess the available evidence for the safety and efficacy of erbium:YAG laser, microablative fractional CO2 laser and RF in the treatment of VVA/GSM. The authors reviewed the current published literature evaluating these therapies. All three therapies appear safe; however, all the studies were uncontrolled and used different protocols and outcome measurements. Therefore, comparison of treatments is difficult. It appears that there is more evidence in favour of the CO2 laser than the erbium:YAG laser. Both lasers have more evidence than RF. In conclusion, microablative CO2 laser, erbium:YAG laser and RF may be offered to patients suffering from VVA/GSM as an alternative or adjunct to conventional therapies. Further well-conducted controlled studies are needed.

Common Benign Chronic Vulvar Disorders.

Common benign chronic vulvar conditions include genitourinary syndrome of menopause (formerly called vulvovaginal atrophy), lichen sclerosus, lichen planus, lichen simplex chronicus, and vulvodynia. Genitourinary syndrome of menopause results from the hypoestrogenic state that leads to atrophy of normal vulvar and vaginal tissues. It is typically treated with lubricants, moisturizers, and intravaginal estrogen. Lichen sclerosus is an inflammatory condition characterized by intense vulvar itching. It is treated with topical steroids or, in some cases, topical calcineurin inhibitors. Patients with lichen sclerosus are at risk of vulvar squamous cell carcinoma and should be monitored closely for malignancy. Lichen planus is an inflammatory autoimmune disorder that can affect the vulva and vagina in addition to other skin and mucosal surfaces. The first-line treatment is topical steroids, and significant scarring can occur if left untreated. Lichen simplex chronicus manifests as persistent itching and scratching of the vulvar skin that leads to thickened epithelium. Breaking the itch-scratch cycle, often with topical steroids, is the key to treatment. Vulvodynia is a common vulvar pain disorder and is a diagnosis of exclusion. A multimodal treatment approach typically includes vulvar hygiene, physical therapy, psychosocial interventions, and antineuropathy medications.

Vulvo-vaginal lichen planus: A focussed review for the clinician.

Vulvo-vaginal lichen planus is a condition which imposes a significant burden of symptoms and sequale. There is a paucity of knowledge and evidenced-based management with significant diagnostic delay prior to appropriate treatment being common. There remains great variability in clinician practice in the context of limited knowledge. This clinical review presents current evidence on the clinicopathological features, practical assessment and management options. Learning points include characterization of this chronic, burdensome clinical entity that has no standardized diagnostic or management protocols. We provide practical clinical conclusions based on current knowledge and identify areas for future research to improve patient outcomes.

Vulval Crohn disease: A case series of 26 patients.

Vulval Crohn disease is rare and likely under-reported, leading to difficulty in diagnosis and delay in treatment. In this case series, we report the clinical features and discuss therapy in 26 consecutive patients with vulval Crohn disease, with or without documented gastrointestinal disease, presenting between January 2016 and July 2018. We highlight the need for treatment that is in some cases more aggressive than the requirement to manage the patient's gastrointestinal involvement.