Plasma Cell Vulvitis: A Classification Into Two Clinical Phenotypes.

OBJECTIVE: The etiology of plasma cell vulvitis (PCV) is debated. The authors aimed to test the hypothesis that PCV could be divided into 2 clinical phenotypes. METHODS: Patients with a clinico-pathological diagnosis of PCV and with available vulvar photos seen in a vulvar clinic were retrospectively studied. The cases of PCV were divided into 2 groups: non-lichen-associated (primary PCV) and lichen-associated (secondary PCV). The 2 groups were compared in terms of age, menopausal status, location of the PCV, and 12 histologic parameters (Fisher exact test, p < .05). RESULTS: Thirty-five patients (20 primary and 15 secondary PCV) were included. The 2 groups did not differ in terms of age (mean, 65; range, 50-85) or menopausal status. Primary PCV was located exclusively on the vestibule for 19/20 patients, whereas secondary PCV was extravestibular for 14 of 15 patients, either exclusively (2) or both extravestibular and vestibular (12). One patient with secondary PCV had solely vestibular involvement. Five histological features were observed significantly more often in case of secondary PCV: epidermal atrophy, parakeratosis, dermal and epidermal neutrophils, and dermal eosinophils. CONCLUSIONS: Plasma cell vulvitis can be divided clinically into 2 phenotypes. Primary non-lichen-associated PCV is restricted to the vestibule and could be the vulvar counterpart of atrophic vaginitis. Secondary lichen-associated PCV is both extravestibular and vestibular, and its clinical and histological features should be looked for outside the PCV areas. This division of PCV into 2 clinical phenotypes could have therapeutic implications.

Nocturnal Vulval Pain in Girls-A Subset of Vulvodynia?

OBJECTIVES: The aims of the authors' case series were to outline the clinical features of prepubertal nocturnal vulval pain syndrome and to look at management and outcomes. METHODS: Clinical details of prepubertal girls experiencing episodes of nocturnal vulval pain with no identifiable cause were recorded and analyzed. Parents completed a questionnaire to look at outcomes. RESULTS: Eight girls with age at onset of symptoms between 3.5 and 8 years (mean 4.4 years) were included. Each patient described intermittent episodes of vulval pain lasting between 20 minutes and 5 hours, starting 1-4 hours after falling asleep. They were crying and rubbing or holding the vulva with no obvious cause seen. Many were not fully awake and 75% had no recollection of the events. Management focused on reassurance alone. The questionnaire showed that 83% had full resolution of symptoms with a mean duration of 5.7 years. CONCLUSIONS: Prepubertal nocturnal vulval pain syndrome may be a subset of vulvodynia (generalized, spontaneous, intermittent) to be included in the clinical spectrum of night terrors. Recognizing the clinical key features should aid prompt diagnosis and reassurance of the parents.

Adult Vulvar Lichen Sclerosus: Can Experts Agree on the Assessment of Disease Severity?

OBJECTIVE: The objective of this study was to test the severity rating of the signs and architectural changes for interrater reliability among world experts via analysis of lichen sclerosus (LS) photographs. METHODS: A recent Delphi consensus exercise established a list of symptoms, signs, and architectural changes, which experts feel are important to include in a severity scale. Photographs of vulvar LS were manually extracted from patient charts and 50 photographs with a range of severity of signs and architectural changes were chosen. Lichen sclerosus experts were invited to take part in the study and 3 dermatologists and 3 gynecologists were selected for their expertise and geographic variety. Raters assessed the photographs for multiple signs and architectural changes as well as an overall impression of disease severity on a 4-point Likert scale. Intraclass correlation coefficients were calculated. RESULTS: The intraclass correlation coefficients were very poor for individual signs and architectural changes as well as for overall disease severity when analyzed for all 6 raters as well as when analyzed with dermatologists' and gynecologists' responses grouped separately. There were no statistically significant correlations found. CONCLUSIONS: Global experts were unable to agree on any signs, architectural changes, or an overall global impression to assess vulvar LS disease severity based on analysis of vulvar photographs. Standardized descriptions regarding what constitutes mild, moderate, and severe signs and anatomical changes are required before further scale development can occur.

The clinical role of LASER for vulvar and vaginal treatments in gynecology and female urology: An ICS/ISSVD best practice consensus document.

BACKGROUND: The clinical role of LASER for vulvar and vaginal treatments in gynecology and female urology is controversial. AIMS: In this best practice document, we propose recommendations for the use of LASER for gynecologic and urologic conditions such as vulvovaginal atrophy, urinary incontinence, vulvodynia, and lichen sclerosus based on a thorough literature review. MATERIALS & METHODS: This project was developed between January and September 2018. The development of this document followed the ICS White Paper Standard Operating Procedures. RESULTS: Most of the available studies are limited by their design; for example they lack a control group, patients are not randomized, follow up is short term, series are small, LASER is not compared with standard treatments, and studies are industry sponsored. Due to these limitations, the level of evidence for the use of LASER in the treatment of these conditions remains low and does not allow for definitive recommendations for its use in routine clinical practice. Histological evidence is commonly reported as proof of tissue regeneration following LASER treatment. However, the histological changes noted can also be consistent with reparative changes after a thermal injury rather than necessarily representing regeneration or restoration of function. The use of LASER in women with vulvodynia or lichen sclerosus should not be recommended in routine clinical practice. There is no biological plausibility or safety data on its use on this population of women. DISCUSSION: The available clinical studies do not present convincing data regarding the efficacy of LASER for the treatment of vaginal atrophy or urinary incontinence. Also, while short-term complications seem to be uncommon, data concerning long-term outcomes are lacking. CONCLUSION: At this point, LASER is not recommended for routine treatment of the aforementioned conditions unless part of well-designed clinical trials or with special arrangements for clinical governance, consent, and audit.

Group A Streptococcal Vulvitis in Adult Women: Clinical Features and Association With Psoriasis.

OBJECTIVES: Group A streptococcus (GAS) vulvitis is rare, mainly reported in association with vaginitis. We examined the clinical features of GAS vulvitis in adults, the presence of other infected sites, and its association with dermatological conditions. MATERIALS AND METHODS: We retrospectively analyzed the medical files and photographs of adult patients with bacteriologically confirmed GAS vulvitis seen at 3 private clinics. Coexisting infected sites, associated dermatological conditions, and bacteriological results for the husbands of 3 patients were examined. RESULTS: Nineteen patients (mean age = 52.2 [range = 23-83] years) with vulvar GAS infections were identified. The vulvar symptoms consisted of pain (11 cases), pruritus (9), burning (4), and discharge (10). The predominant physical feature was bilateral erythema, mainly located on the labia minora and the inner aspect of the labia majora. This erythema was associated with oozing (3), edema (6), or fissures (6). Seventeen patients had an associated vaginal infection, which was asymptomatic in 7 cases; anal infections were present in 9 cases. The following 10 patients had associated dermatological conditions: psoriasis (6), lichen sclerosus (2), Paget disease (1), or vitiligo (1). Two (one each from the throat and penis) of the 3 bacteriological specimens taken from the 3 husbands were GAS positive. CONCLUSIONS: In most adult women, GAS vulvitis is associated with a vaginal infection that may be asymptomatic. A bilateral, oozing, and edematous or fissured erythema involving the vulvar or anovulvar area is suggestive of GAS vulvitis. The association with psoriasis and the benefits of screening household members and sexual partners deserves further attention.

Descriptors of Vulvodynia: A Multisocietal Definition Consensus (International Society for the Study of Vulvovaginal Disease, the International Society for the Study of Women Sexual Health, and the International Pelvic Pain Society).

OBJECTIVES: Three scientific societies, the International Society for the Study of Vulvovaginal Disease (ISSVD), the International Society for the Study of Women Sexual Health (ISSWSH), and the International Pelvic Pain Society (IPPS) developed the "2015 ISSVD, ISSWSH, and IPPS Consensus Terminology and Classification of Persistent Vulvar Pain and Vulvodynia" (referred to as the "2015 consensus terminology").The terminology included 11 descriptors of vulvodynia. However, the definitions of the descriptors were not included in the 2015 consensus terminology publications. The objective of this article was to provide these definitions. MATERIALS AND METHODS: The ISSVD led a discussion on the definitions for the 11 vulvodynia descriptors, with participation from the ISSWSH and IPPS. The definitions were created through a consensus process. RESULTS: The definitions are described and the rationale for their choice is elucidated. CONCLUSIONS: The definitions of vulvodynia descriptors were determined by a multistaged process of discussion among health care providers with expertise in the pathophysiology, evaluation, and treatment of vulvodynia. The definitions were approved by the ISSVD, ISSWSH, and IPPS. It is recommended that these definitions of vulvodynia descriptors as well as the 2015 consensus terminology be used for the classification of vulvodynia.

The Clinical Role of LASER for Vulvar and Vaginal Treatments in Gynecology and Female Urology: An ICS/ISSVD Best Practice Consensus Document.

In this best practice document, we propose recommendations for the use of LASER for gynecologic and urologic conditions such as vulvovaginal atrophy, urinary incontinence, vulvodynia, and lichen sclerosus based on a thorough literature review. Most of the available studies are limited by their design; for example, they lack a control group, patients are not randomized, follow-up is short term, series are small, LASER is not compared with standard treatments, and most studies are industry sponsored. Because of these limitations, the level of evidence for the use of LASER in the treatment of these conditions remains low and does not allow for definitive recommendations for its use in routine clinical practice. Histological evidence is commonly reported as proof of tissue regeneration after LASER treatment. However, the histological changes noted can also be consistent with reparative changes after a thermal injury rather than necessarily representing regeneration or restoration of function. The use of LASER in women with vulvodynia or lichen sclerosus should not be recommended in routine clinical practice. There is no biological plausibility or safety data on its use on this population of women. The available clinical studies do not present convincing data regarding the efficacy of LASER for the treatment of vaginal atrophy or urinary incontinence. Also, although short-term complications seem to be uncommon, data concerning long-term outcomes are lacking. Therefore, at this point, LASER is not recommended for routine treatment of the aforementioned conditions unless part of well-designed clinical trials or with special arrangements for clinical governance, consent, and audit.

International Society for the Study of Vulvovaginal Disease Recommendations Regarding Female Cosmetic Genital Surgery.

Female genital cosmetic surgeries (FGCSs) and procedures are increasingly being advertised as common, simple, and complication-free, capable of not only improving aesthetic appearance but also increasing self-esteem and sexual pleasure.Guidelines for physicians and clear, scientifically correct information for patients must be made available, to minimize the number of ineffective or deleterious procedures.The International Society for the Study of Vulvovaginal Disease positions/recommendations regarding FGCS are as follows:1. There is a wide variation regarding genital normalcy; providers must be able to explain this to women.2. There are no data supporting FGCS including, G-spot augmentation, hymenoplasty, vulvar and perianal bleaching/whitening, vaginal tightening procedures, and other procedures aimed at increasing sexual function.3. Women should not be offered FGCS before the age of 18 years.4. Women undergoing FGCS should be evaluated by a provider with expertise in vulvovaginal diseases, including attention to their psychological, social, and sexual context. Evaluation by an experienced mental health provider should be considered when the motivation for seeking surgery and/or expectations are not clear or realistic.5. Female genital cosmetic surgery is not exempt from complications.6. Informed consent must always be obtained.7. Surgeons performing FGCS should refrain from solicitous advertising or promoting procedures without scientific basis, including on Web sites.8. Surgeons should not perform surgery that they do not agree with and explain their rationale/position when pressured by patients.9. The genital surgeon must be adequately trained in performing FGCS including knowledge of the anatomy, physiology and pathophysiology of the vulva, vagina and adjacent organs.

Vulvar hidradenoma papilliferum (HP) is located on the sites of mammary-like anogenital glands (MLAGs): Analysis of the photographs of 52 tumors.

BACKGROUND: Hidradenoma papilliferum (HP) is a benign tumor that primarily affects the anogenital area of adult women. Previously considered apocrine tumors, anogenital HP tumors are now interpreted as adenomas of mammary-like anogenital glands based on their histologic features. OBJECTIVE: This clinical study was undertaken to determine whether vulvar HP is located on mammary-like anogenital gland sites and to describe its morphologic features. METHODS: The clinical photographs of 52 histologically confirmed vulvar HP provided by 7 vulva specialists were analyzed. RESULTS: In all, 90.4% of the HP were located on the interlabial sulcus, adjacent zone, or the perineum. These tumors were polymorphous in terms of number (1 or multiple), size (<1-4.5 cm), color (pink, red, blue), surface (ulcerated or not), and architecture (unilobular or multilobular). LIMITATIONS: Eight histologic reports could not be reviewed by the authors but the contributors confirmed that the photographs sent were only those of histologically confirmed HPs. CONCLUSIONS: Vulvar HP is mainly located on mammary-like anogenital gland sites, thereby providing further evidence to their histogenesis. Although a nonulcerated or ulcerated tumor of the interlabial sulcus should evoke a HP diagnosis, the latter must be confirmed histologically.

Unilateral vulval swelling in cyclists: a report of 8 cases.

OBJECTIVE: To define the clinical features, imaging results, histopathologic patterns, and clinical outcomes seen in patients who develop vulval swelling as a result of intensive cycling. METHODS: The case notes of 8 female cyclists were retrospectively reviewed. RESULTS: The mean age of the patients was 45 years, and all were cycling long distances each week (range, 125-450 km; median, 210 km). All patients had a unilateral swelling of the labium majus. Five patients had magnetic resonance imaging showing asymmetry of the vulva with no enhanced signaling associated with inflammation. Histologic examination in 3 cases revealed dermal fibrosis and dermal edema associated with dilatation of lymphatic vessels. In 1 case, a perifollicular inflammatory infiltrate containing epithelioid granulomas was seen but Crohn's disease was excluded. In another case, fibromuscular hyperplasia was seen. Six patients had surgical excision of the swollen area, and 4 patients were able to resume cycling without problems up to 5 years of follow-up. The cosmetic appearance was initially considered satisfactory by 5 patients. However, 2 patients required a second surgical procedure to further improve the cosmetic appearance. In 1 case, there was a postoperative hematoma followed by an immediate recurrence of the swelling, which persisted. CONCLUSIONS: Unilateral swelling of the labium majus occurring in cyclists can be related to dermal fibroedema associated with lymphatic dilatation or to an increase in adipose tissue. Surgical results were satisfactory in 5 of the 6 patients who underwent excision.

Are virtual consultations suitable for patients with vulval disease? A multicentre audit of outcomes in the COVID-19 pandemic.

Background: During the COVID-19 pandemic, virtual consultation (VC) was used to replace in-person consultations. This raises specific questions when dealing with vulval conditions. Objectives: To assess the feasibility and the efficiency of VC with and without supplementary imaging, in patients with vulval conditions, and to evaluate the images provided as an aid to diagnosis. Methods: This prospective multicentre audit took place in three specialized vulval clinics in London and Paris. Anonymized data on patients' clinical characteristics, consultation characteristics (including the number and quality of any supplementary images provided) and consultation outcomes (diagnostic certainty and physician satisfaction) were collected. Characteristics and outcomes in those with or without supplementary imaging were compared amongst both new and follow-up consultations. Results: A total of 316 VCs were included. In total, 18.7% (n = 59) were new patient consultations and 81.3% (n = 257) were follow-up. Supplementary imaging (photographs and/or video recordings) were provided by 28.5% (n = 90) of the total cohort. Median photographic quality was significantly higher on a five-point Likert-type scale when photographs were taken by a third party as opposed to the patient themselves (4 vs. 3, Mann-Whitney U-test, p < 0.0001). There was no association between the provision of supplementary imaging and diagnostic certainty amongst new patient consultations. However, a higher proportion of follow-up patients who provided supplementary imaging received definitive management decisions (χ 2 test, p < 0.001) and physician satisfaction with these consultations, as measured on a five-point Likert-type scale, was significantly higher (Mann-Whitney U-test, p < 0.0001). Furthermore, median physician satisfaction scores ≥4 were observed in follow-up consultations for candidiasis, lichen simplex/eczema and vulvodynia. Conclusions: Although in-person consultation remains the gold standard of care, VC may have a role in the management of selected patients with vulval disease. It is possible to provide good-quality photographs for clinical assessment, particularly with the help of a third party and follow-up patients with an established, cancer-unrelated diagnosis may be best suited for this consultation modality.

Incomplete efficacy of 5-aminolevulinic acid (5 ALA) photodynamic therapy in the treatment of widespread extramammary Paget's disease.

Photodynamic therapy (PDT) using 5-aminolevulinic acid (5-ALA) is an effective treatment for several conditions such as Bowen's disease, subsets of basal cell carcinomas and actinic keratosis. Surgical resection is the first-choice therapy for extramammary Paget's disease (EMPD), but extensive resection is highly invasive and recurrences are frequent. We report two cases of genital EMPD treated by PDT with partial efficacy. The first patient, a 78-year-old male, suffered from pubic and scrotal Paget's disease for 6 years despite numerous treatments. The second patient, a 78-year-old female, had vulvar involvement for 2 years that was resistant to multiple treatments. The disease was recurrent and chronic with important pruritus and significant impact on the quality of life. Methyl 5-aminolevulinate was applied for 3 h, and irradiation was applied with red light (630 nm) using a total light dose of 37 J/cm(2) for a period of 10 min. The patients were treated every 2 to 4 weeks for a total of at least three treatments. Both patients experienced a partial transient reduction in their symptoms. One patient had a partial transient remission (< 50% reduction of the involved surface), whereas in the other patient, PDT failed to reduce the surface area of the lesions.

2011 ISSVD Terminology and classification of vulvar dermatological disorders: an approach to clinical diagnosis.

OBJECTIVE: The study aimed to formulate an easy clinical approach that may be used by clinicians of all backgrounds to diagnose vulvar dermatological disorders. MATERIALS AND METHODS: The International Society for the Study of Vulvovaginal Disease appointed a committee with multinational members from the fields of dermatology, gynecology, and pathology and charged the committee to formulate a clinically based terminology and classification of vulvar dermatological disorders. The committee carried out its work by way of multiple rounds of e-mails extending over almost 2 year's time. RESULTS: The committee was able to formulate a consensus report containing terminology, classification, and a step-wise approach to clinical diagnosis of vulvar dermatological disorders. This report was presented and approved by the International Society for the Study of Vulvovaginal Disease at the XXI International Congress held in Paris, France, on September 3 to 8, 2011. CONCLUSIONS: The authors believe that the approach to terminology and classification as well as clinical diagnosis contained in this article allows clinicians to make highly accurate diagnoses of vulvar dermatological disorders within the clinical setting. This, in turn, will reduce the need for referrals and will improve the care for women with most vulvar disorders.

Vulvar pseudotumoral acyclovir-resistant herpes in an HIV-negative, non-immunosuppressed patient: A therapeutic challenge.

BACKGROUND: Vulvar pseudotumoral herpes infections have been reported in HIV-positive patients. A 32-year-old HIV-negative woman presented with a 6-month history of a vulvar pseudotumor that had been unresponsive to oral acyclovir and valacyclovir, as well as topical imiquimod. OBJECTIVE: This study aimed to evaluate the therapeutic efficacy of a multidrug regimen for vulvar pseudotumor herpes infection in an HIV-negative patient. METHODS: Histology revealed multinucleated giant cells, consistent with a herpes infection. The patient's herpes simplex virus type 2 was resistant to acyclovir. Immunomodulatory agents (thalidomide and topical imiquimod) were started. RESULTS: The lesion enlarged after 6 weeks of treatment. Topical cidofovir 1% gel was added. There was gradual decrease in the pseudotumor size. After 7 months, the Pseudotumor had resolved. CONCLUSION: This is the first reported case of vulvar pseudotumoral herpes in an immunocompetent, HIV-negative patient. Oral thalidomide, in association with topical imiquimod and topical cidofovir, was effective in treating acyclovir-resistant pseudotumoral herpes of the vulva.

Epidemiological and economic burden of potentially HPV-related cancers in France.

Human papillomaviruses (HPV) infection is now known to be responsible for almost all cervical cancers, and for a substantial fraction of Head and Neck cancers (HNCs). However, comprehensive epidemiological and economic data is lacking in France, especially for rarer potentially HPV-related cancers, which include anal, vulvar and vaginal cancers. Using the national comprehensive database of French public and private hospital information (PMSI), we assessed prevalence and incidence of patients with in-hospital diagnosis for potentially HPV-related cancers in 2013, and estimated costs related to their management over a 3-year period after diagnosis in France. Concerning female genital cancers, 7,597, 1,491 and 748 women were hospitalized for cervical, vulvar and vaginal cancer in 2013, respectively, with 3,120, 522 and 323 of them being new cases. A total of 4,153 patients were hospitalized for anal cancer in 2013, including 1,661 new cases. For HNCs, 8,794 and 14,730 patients were hospitalized for oral and oropharyngeal cancer in 2013, respectively; 3,619 and 6,808 were new cases. Within the 3 years after cancer diagnosis, the average cost of hospital care per patient varied from €28 K for anal cancer to €41 K for oral cancer. Most expenditures were related to hospital care, before outpatient care and disability allowance; they were concentrated in the first year of care. The total economic burden associated with HPV-potentially related cancers was about €511 M for the French National Health Insurance over a 3 years period (2011 to 2013), ranging from €8 M for vaginal cancer to €222 M for oropharyngeal cancer. This study reported the most up-to-date epidemiological and economic data on potentially HPV-related cancers in France. These results may be used to evaluate the potential impact of new preventive strategies, namely the generalized organized screening of cervical cancer and the nine-valent HPV vaccine, indicated in the prevention of cervical, vaginal, vulvar and anal cancers.

2006 ISSVD classification of vulvar dermatoses: pathologic subsets and their clinical correlates.

The International Society for the Study of Vulvovaginal Disease (ISSVD) has, as one of its major societal goals, the development and promulgation of nomenclature and classification of vulvar disease. A committee of the ISSVD has developed new nomenclature and classification for the specific area of vulvar dermatoses. This classification was approved by the ISSVD members at the most recent international congress, held in New Zealand in February 2006.

Spontaneous regression of grade 3 vulvar intraepithelial neoplasia associated with human papillomavirus-16-specific CD4(+) and CD8(+) T-cell responses.

Cell-mediated immunity directed against human papillomavirus 16 (HPV-16) antigens was studied in six patients affected with grade 3 vulvar intraepithelial neoplasia (VIN3, also known as bowenoid papulosis). Five of the patients presented with a chronic and persistent disease that relapsed after destructive treatments. They showed no detectable anti-HPV blood T-cell responses and no T-cell intraepidermal vulvar infiltrate containing both CD4+ and CD8+ lymphocytes. The last patient had a complete clearance of viral lesions, 8 months after disease onset and 2 months after electrocoagulation of <50% of the VIN3 lesions. She showed high frequency anti-E6 and anti-E7 effector blood T cells by ex vivo ELISpot-IFNgamma assay before clinical regression. Immunohistochemical study of her vulvar biopsy revealed a marked dermal infiltrate containing a majority of CD4+ T lymphocytes and an epidermal infiltrate made up of both CD4(+) and CD8(+) T cells. This seems to be the first evidence of an association between spontaneous regression of VIN3 lesions and HPV-specific T-cell responses detectable in the blood. Hence, an increase of HPV-specific effector T lymphocyte responses by vaccine-based therapeutic strategies might be useful to clear the lesions in bowenoid papulosis disease.