Plasma Cell Vulvitis: A Case Series and Follow-Up Study.

OBJECTIVES: Plasma cell vulvitis (PCV) is a rare inflammatory vulvar condition. The aim of this study was to describe the natural history, treatment, impact on quality of life, and factors associated with poorer outcomes for PCV. METHODS: A mixed-methods approach was used combining a retrospective case note review with a cross-sectional telephone questionnaire. All women diagnosed with PCV attending the vulvar disorders clinic at the Royal Women's Hospital between January 2011 and December 2020 were included. RESULTS: During the 10-year study period, 7,500 women were seen at the vulval disorders clinic, of whom 21 were diagnosed with PCV (0.28%). Of these women, 12 who were followed up for more than 12 months agreed to participate in the study. At a median of 5 years follow-up, there was variability in symptom severity, with more than half of the women still symptomatic with pain, precipitated by friction and dyspareunia, resulting in a moderate to large impact on quality of life. There were 5 women who were asymptomatic. Only 1 woman had a preexisting history of lichen planus and lichen sclerosus. Potent topical corticosteroids were identified as the preferred treatment. CONCLUSIONS: Women with PCV can remain symptomatic for many years with significant impacts on quality of life, which may consequently require long-term support and follow-up.

Management of severe vulval lichen sclerosus with adalimumab.

Vulval Lichen Sclerosus (VLS) is a chronic inflammatory dermatosis that significantly impacts the quality of life. We report 2 cases of severe treatment-resistant VLS treated with subcutaneous adalimumab. One patient showed modest clinical improvement with no significant improvement in quality-of-life measures. The second patient showed a significant improvement in both clinical and quality-of-life measures. Both patients achieved optimal control with a maintenance dosing of 40 mg subcutaneously weekly. Adalimumab may be a treatment option for recalcitrant VLS; however, clinical trials are required to confirm efficacy for this indication.

Isotretinoin prescribing and monitoring practices among Australian dermatologists measured against current guidelines, and identification of practice uncertainties.

This letter summarizes the outcomes from an anonymous survey conducted among Australian dermatologists. It provides a comparison of Australian isotretinoin prescribing practices against best available evidence and guidelines in the UK, Europe and USA.

Plasma Cell Vulvitis: A Systematic Review of Interventions.

OBJECTIVES: Plasma cell vulvitis (PCV) is a rare chronic inflammatory disorder, where the symptoms can be severe and may affect patient's quality of life. However, there are currently no evidence-based treatment guidelines. The aims of this systematic review were to evaluate efficacy of individual treatments and to inform future research. MATERIALS AND METHODS: A systematic search was conducted of publication between 1952 and August 2020 via MEDLINE, Embase, and Emcare. All publications that evaluated the efficacy of treatments for patients with PCV were included. Forty-seven publications comprising 45 case reports and case series and 2 cohort studies were included. RESULTS: To date, there are no randomized controlled trials evaluating the efficacy of different treatment options. There are also no studies that assess the impact of treatment on quality of life. Topical corticosteroids are the most frequently used first-line therapy, with limited evidence to support their efficacy, followed by topical imiquimod and surgical excision. The least supported intervention is cryotherapy. CONCLUSIONS: Although descriptive studies support the use of topical corticosteroids and, to a lesser degree, topical calcineurin inhibitors and imiquimod, this review highlights the need for standardized outcome measures and randomized clinical trials for more definitive therapeutic recommendations for women with PCV.

Vulval lichen sclerosus: An Australasian management consensus.

BACKGROUND/OBJECTIVES: Vulval lichen sclerosus (VLS) is a chronic inflammatory skin condition predominantly affecting the anogenital region in women and children. To date, there is lack of agreement amongst experts on a severity scale to aid assessment, research and treatment stratification on VLS. Furthermore, literature on best practice for long-term management of VLS is lacking. The aim of this consensus is to provide broad guidelines on the short and long-term management of VLS. METHODS: An initial focus group of Australasian experts in vulval dermatology developed a draft consensus statement for the management of VLS. Based on the results of the draft statement, a consensus panel of 22 Australasian experts, comprised of the initial and additional members, participated in an anonymous four-stage eDelphi process. Round 1 involved generation and voting on statements from the draft consensus statement developed by the focus group. In Rounds 2, 3 & 4, panel members were presented formal feedback from previous rounds and asked to indicate their level of agreement. Consensus was reached if there was ≥70% agreement on the importance of an item in the 4 (agree) to 5 (strongly agree) range. RESULTS: The expert panel, with a total of 504 collective years of experience in the field of VLS, reached consensus on a core set of 51 management statements related to diagnosis, severity, initial and long-term management, follow-up, and complications of VLS. CONCLUSIONS: This study has identified a set of management statements for VLS that may be useful in clinical practice in the Australasian population.

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.

Outcome Measurement Instruments for Provoked Vulvodynia: A Systematic Review.

OBJECTIVE: The objective of this study was to detail the outcome measurement instruments used in randomized control trials and observational studies investigating therapeutic interventions for provoked vulvodynia. MATERIALS AND METHODS: We searched Ovid Medline, Embase, Emcare, and PyschINFO libraries from database inception through April 2017. We included randomized control trials and observational studies of provoked vulvodynia that used instruments to measure the outcome of therapeutic interventions. RESULTS: A total of 2299 articles were retrieved and 25 were eligible for inclusion in accordance with the selection criteria. The included studies measured 26 different outcomes, using 110 outcome measurement instruments. Patient-reported outcomes were most commonly measured (144/166, 86%), followed by physician-reported outcomes (20/166, 12%). The most commonly measured outcomes were patient-reported psychological impact of disease (27/166, 16%), patient-reported improvement in dyspareunia (25/166, 15%), and patient-reported reduction in pain (24/166, 14%). The Pain Catastrophizing Scale, the Beck Depression Inventory, and the State Trait Anxiety Questionnaire were the most commonly used instruments to measure psychological impact.The most commonly measured clinician-rated outcome was an improvement in pain (17/166, 10%), which was most frequently assessed by the cotton swab test. Only 34 (31%) outcome measurement instruments were specific to vulvodynia (26/110, 23%) or sexual functioning (8/110, 7%). CONCLUSIONS: There is a wide range of outcome measurement instruments used in provoked vulvodynia studies, resulting in inconsistency of reporting and difficulty in comparing and combining findings for systemic review. There is a pressing need for the development of validated, reliable instruments and consensus on a core outcome set for further research purposes.