Attitudes Toward Proactive Topical Corticosteroid Use Among Women With Vulval Lichen Sclerosus.

OBJECTIVES: Some practitioners are adopting proactive topical corticosteroid (TCS) therapy for vulval lichen sclerosus (VLS). We sought to understand patient attitudes toward proactive TCS therapy for VLS in a context in which proactive therapy is adopted. METHODS: Four online focus group discussions with 12 participants. Data analysis was informed by social constructionist grounded theory. RESULTS: All participants had accepted a proactive regimen. Three themes were developed from the analysis: "Coming to accept proactive therapy," "Motivators to maintaining a proactive regimen," and "The importance of a routine that fits me." Within each theme are subthemes illustrating different dimensions of the theme. CONCLUSIONS: Accepting proactive TCS therapy for VLS requires incorporating regular TCS use into a patient's identity, unlearning previous understandings regarding the safety of long-term TCS use, and adopting a regimen that fits within patients' lives and minimizes the loss of autonomy.

The efficacy and safety of deucravacitinib compared to methotrexate, in patients with vulvar lichen planus who have failed topical therapy with potent corticosteroids: a study protocol for a single-centre double-blinded randomised controlled trial.

BACKGROUND: Vulvar lichen planus (VLP) is a chronic vulvar dermatosis that is difficult to treat and can severely impair quality of life in the absence of adequate treatment. There is a lack of high-quality evidence to direct therapy for VLP. This randomised controlled trial will be the first double-blinded study comparing systemic treatments in VLP and aims to investigate the safety and efficacy of deucravacitinib compared to methotrexate, in patients with VLP who have failed treatment with potent topical corticosteroids. METHODS: A total of 116 women aged ≥ 18 years with moderate to severe VLP (Genital Erosive Lichen Planus (GELP) score ≥ 5) will be recruited. All participants will initially be treated with Diprosone® OV daily, and their outcome will be assessed using the GELP score. At 8 weeks' follow-up, responders (GELP < 5) will be continued on Diprosone® OV. Non-responders (GELP ≥ 5) will be randomised 1:1 in a blinded fashion to receive (i) methotrexate 10 mg weekly + placebo tablet twice daily + folic acid 5 mg weekly or (ii) deucravacitinib 6 mg twice daily + placebo tablet weekly + folic acid 5 mg weekly. The primary endpoint is the difference in the mean change of GELP scores from baseline to week 32 between deucravacitinib and methotrexate groups. DISCUSSION: High-quality evidence guiding the management of women with VLP is lacking. Once completed, this will be the first double-blinded RCT to compare systemic treatments in VLP. The results of this study will provide valuable, high-quality data to guide second-line therapy options for VLP that is recalcitrant to potent topical corticosteroids. TRIAL REGISTRATION: Australian New Zealand Clinical Trials Registry ACTRN12623000682640. Registered on 26 June 2023.

Adult genital psoriasis: An updated review for clinicians.

Genital psoriasis is a chronic inflammatory skin condition that has been reported in up to 63% of patients with psoriasis on other parts of their skin. It has a profound impact on quality of life and sexual function which is often overlooked by current severity scores. Despite its prevalence and disease burden, genital psoriasis remains largely under-reported and under-treated. Historically, this was due to the impracticality and limited efficacy data of standard psoriasis treatments when applied to genital skin. However, there have been recent advancements with several new agents currently being developed and evaluated for genital psoriasis. This clinical review aims to provide an overview of the current evidence regarding the clinical features of genital psoriasis, available management options and tools for assessing patients' quality of life. Key takeaways from this review emphasise the recognition of genital psoriasis as a chronic and debilitating condition, unique in its impact on patients' quality of life, necessitating sensitive and attentive approaches to address their needs.

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.

Why do some patients with vulval lichen sclerosus on long-term topical corticosteroid treatment experience ongoing poor quality of life?

OBJECTIVE: To identify reasons for ongoing poor quality of life (pQOL) in a subset of long-term topical corticosteroid-treated vulval lichen sclerosus (VLS) patients. METHODS: A prospective cross-sectional study of patients attending a dermato-gynaecology practice in Sydney, Australia, comparing VLS patients with good quality of life (gQOL) and pQOL, in pre-treatment and long-term treatment groups, using the Vulval Quality of Life Index (VQLI). Demographics, VQLI scores and treatment characteristics were compared between gQOL and pQOL patients. RESULTS: A total of 255 biopsy-proven VLS patients, 67 in pre-treatment and 188 in long-term treated groups were considered. There were 33 (49.3%) pQOL patients in pre-treatment and 13 (6.9%) in treatment groups (p < 0.001). The highest-scoring domain in treated pQOL patients was sexuality (1.7 [interquartile range (IQR) 1.0-2.0]), followed by anxiety [1.3 (IQR 1.0-1.5]), symptoms (1.0 [IQR 0.5-1.5]) and activities of daily living (0.7 [IQR 0.3-1.0]). Compared to treated gQOL, treated pQOL had significantly higher proportions of patients with partial treatment adherence (8 [61.5%] vs 42 [24.0%], p = 0.006), suboptimal disease control (7 [53.8%] vs 20 [11.4%], p < 0.001), scarring progression (3 [23.1%] vs 7 [4.0%], p = 0.024) and urinary incontinence (5 [38.5%] vs 27 [15.4%], p = 0.049). CONCLUSIONS: Only a minority of long-term treated VLS patients reported ongoing pQOL. Of those who did, sexuality and anxiety domains were found to be the main sources of distress. Three major areas distinguishing gQOL from pQOL patients were (1) treatment adherence and disease control, (2) psychological factors and (3) urinary incontinence.

Highlighting adalimumab as a treatment option for systemic treatment of toxic epidermal necrolysis: A case series from a tertiary specialised burns centre.

Toxic epidermal necrolysis and Stevens-Johnson Syndrome describe a spectrum of severe cutaneous skin reactions constituting a medical emergency, and no formal treatment guidelines exist to direct systemic immunosuppressive therapy although referral to a burns unit and wound management remains a mainstay of treatment. We performed a retrospective chart review on all patients at a single centre with TEN between 2017 to 2021 to compare clinical characteristics and outcomes of those treated with the tumour necrosis factor-alpha (TNF-α) inhibitor adalimumab against non-TNF-α immunosuppressants such as glucocorticoids, intravenous immunoglobulin and cyclosporine. All patients treated with adalimumab had successful resolution of their TEN, resulting in a mean duration of hospital admission of 22.5 days compared to 33 days for patients treated with non-TNF-α inhibitors. We highlight adalimumab as a promising systemic immunomodulator in the treatment of TEN with efficacy comparable to other immunosuppressive agents and associated with a shorter duration of hospital admission.

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.

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.

Acute Vulvar Aphthous Ulceration After COVID-19 Vaccination: 3 Cases.

OBJECTIVE: We present a case series of acute vulvar aphthosis immediately following COVID-19 vaccination. MATERIALS AND METHODS: We describe 3 cases of acute vulvar aphthosis following Pfizer Comirnaty BNT162b2 mRNA and AstraZeneca (Vaxzevria) ChAdOx1 nCoV-19 COVID-19 vaccination in adolescent girls. RESULTS: All patients developed vulvar aphthosis within a few days after receiving COVID-19 vaccination. The onset of vulvar aphthosis was observed to correlate with the dosing schedule known to produce the highest likelihood of adverse effects, first dose in AstraZeneca (Vaxzevria) ChAdOx1 nCoV-19 and second dose in Pfizer Comirnaty BNT162b2 mRNA COVID-19 vaccine. Two patients required oral prednisolone and hospital admission for indwelling urinary catheterization due to urinary retention. Full disease resolution with no sequalae was achieved in all three patients. CONCLUSIONS: Clinicians should be aware of the possible risk of vulvar aphthosis after COVID-19 vaccine administration. Nevertheless, its occurrence should not prevent affected patients from receiving future doses of COVID-19 vaccines, as the mortality and morbidity of COVID-19 infection significantly outweigh the risk of vulvar aphthosis recurrence.

Successful treatment of vulvovaginal lichen planus with tildrakizumab: A case series of 24 patients.

Vulvovaginal lichen planus (VLP) is a chronic inflammatory dermatosis affecting the genital skin and mucosa that can have a profound negative impact on patient quality of life. Up to 43% of women with VLP require systemic immunosuppression to achieve disease remission, and some individuals prove to be highly treatment resistant. We present a case series of 24 women with severe VLP who successfully achieved remission using off-label treatment with the interleukin-23 (IL-23) monoclonal antibody blocker tildrakizumab, and highlight tildrakizumab as a treatment for women with recalcitrant VLP who have failed more conservative treatments.

Does compliance to topical corticosteroid therapy reduce the risk of development of permanent vulvar structural abnormalities in pediatric vulvar lichen sclerosus? A retrospective cohort study.

BACKGROUND: Vulvar lichen sclerosus (VLS) is a chronic inflammatory dermatosis of the genital skin, with up to 20% of cases in the pediatric age group. Limited data exist concerning the prognosis of pediatric VLS, particularly the likelihood of permanent architectural change and whether this can be prevented by compliance with topical corticosteroid treatment (TCS). OBJECTIVE: To evaluate the extent to which compliance to TCS treatment influences the risk of developing vulvar structural abnormalities, including clitoral phimosis and diminutive or fused labia minora. METHODS: A retrospective chart review of case records of pediatric-age females with VLS between January 31, 2004 and January 31, 2021. RESULTS: One hundred eighteen cases of VLS were identified, with a mean age at diagnosis of 7.25 years and a mean follow-up period of 42.7 months. Thirty-four girls were "partially compliant," whereas 84 were "compliant." The risk ratio (RR) of developing any vulvar structural abnormality or clitoral phimosis was 5.76 (95% CI 2.96-11.3) and 21.2 (95%CI 5.23-85.9) times higher, respectively, in partially compliant compared with compliant subjects. The RR of a partially compliant female with pre-pubertal onset VLS having a vulvar structural abnormality persisting beyond menarche was increased 3.54-fold relative to compliant females (95% CI 1.75-7.17). LIMITATIONS: The retrospective nature of our data, lack of a control group, wide variability in follow-up duration, and nonstandardized method of stratifying compliance. CONCLUSION: Vulvar structural abnormalities are common in prepubertal onset VLS. Compliance to TCS appears to be critical in the prognosis of pediatric VLS although attitudes underpinning noncompliance to TCS treatment require further elucidation.

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.

Quality of Life in Vulvar Lichen Sclerosus Patients Treated With Long-Term Topical Corticosteroids.

OBJECTIVE: The aim of the study was to investigate the quality of life in vulvar lichen sclerosus (VLS) patients treated with long-term, individualized topical corticosteroids. METHODS: A prospective cross-sectional study comparing new pretreatment and long-term treated VLS patients attending a dermatogynecology practice in Sydney, Australia. Participants were invited to complete the Vulvar Quality of Life Index (VQLI). The VQLI scores were compared between the 2 groups. RESULTS: A total of 204 biopsy-proven VLS patients-68 new pretreatment and 136 treated patients on topical corticosteroids for 2 years or longer. Treated participants scored lower in all VQLI components, including total score (median = 2.0 [interquartile range {IQR} = 0.0-6.0] vs 13.5 [IQR = 7.5-22.0]; p < .001), symptoms (median = 0.5 [IQR = 0.0-0.5] vs 1.3 [IQR = 0.8-2.0]; p < .001), anxiety (median = 0.0 [IQR = 0.0-0.3] vs 0.8 [IQR = 1.1-2.0]; p < .001), activities of daily living (median = 0.2 [IQR = 0.0-0.3] vs 0.5 [IQR = 0.2-1.1]; p < .001), and sexuality (median = 0.0 [IQR = 0.0-0.7] vs 1.0 [IQR = 0.0-2.0]; p < .001). A higher proportion of treated patients achieved total scores of 0-5, representing nil to minimal impact of VLS on quality of life (98 [72.1%] vs 8 [11.8%]; p < .001). Mild and reversible adverse effects were developed in 11 patients (8.1%). Partially compliant patients were 12 times as likely to develop scarring progression than fully compliant patients (7 [22.6%] vs 2 [1.9%]; p < .001). CONCLUSIONS: Long-term, individualized topical corticosteroid treatment is safe and effective in maintaining disease remission and improves the quality of life of VLS patients. Fully compliant patients demonstrate better treatment outcomes than partially compliant patients.

Relationship between PASI and FDLQI in paediatric psoriasis, and treatments used in daily clinical practice.

BACKGROUND/OBJECTIVES: Psoriasis is a chronic, inflammatory skin disease that affects 0.71% of children. Skin diseases can have a significant impact on quality of life not only for the children affected by psoriasis, but also for their parents and carers. This study aimed to achieve more insight into the quality of life (QOL) of family of paediatric patients with psoriasis, and to investigate whether disease severity scores correlate with Family Dermatology Life Quality Index (FDLQI) scores. In addition, we also observed the treatments used to treat paediatric psoriasis in daily clinical practice. METHODS: Patients with paediatric psoriasis aged 16 and under who visited our outpatient department were included. Baseline Psoriasis Area and Severity Index (PASI) and FDLQI were measured and analysed. RESULTS: Of 157 patients were included in the study. Median PASI was 4.2 (IQR 2.6-6.9), and the median FDLQI was 12 (IQR 7-17). The correlation coefficient between PASI and FDLQI was 0.44 (P < 0.001). Burden of care was the highest scoring item on the FDLQI, followed by emotional impact. 146 patients were treated with topical therapy with a mean improvement in PASI of 3.92. 19 patients underwent phototherapy, and 19 underwent systemic therapy. CONCLUSIONS: In this largest study to date studying the impact of PASI on FDLQI, disease severity was found to be strongly associated with adverse quality of life of family members of paediatric psoriasis patients.

Quality of life of women with untreated vulval lichen sclerosus assessed with vulval quality of life index (VQLI).

BACKGROUND/OBJECTIVE: The diagnosis of vulval lichen sclerosus (VLS) is often delayed, and little is known about quality of life (QoL) of women with it prior to receiving diagnosis and treatment. This study aimed to investigate the impact on QoL on patients with previously untreated VLS using the Vulval Quality of Life Index (VQLI). METHODS: A prospective cross-sectional survey of patients attending a dermato-gynaecology practice in Sydney, Australia from March 2018 to November 2019. Patients with a new biopsy-proven diagnosis of VLS not previously commenced on topical corticosteroid treatment were invited to complete the VQLI. RESULTS: A total of 68 participants with median age of 58 (interquartile range, IQR 48-67) years. Median symptom duration was 24 (IQR 11-60) months. Scarring was present in 53% of participants. The median total score was 13.5 (IQR 7.5-22.0), global score 1.0 (IQR 1.0-2.0). Twelve per cent had nil to minimal effect on QoL, 38% had a mild effect on QoL, 28% had a moderate effect on QoL, 22% had a severe effect on QoL, and none had a very severe effect on QoL. The highest-scoring domains were symptoms, followed by sexuality, anxiety and activities of daily living. CONCLUSION: There was significant impairment in all QoL areas of women with untreated VLS. Most experienced moderate-to-severe impairment as a result of the disease and approximately half had already developed scarring at the time of diagnosis. The findings emphasise the importance of improved awareness, early diagnosis and early commencement of treatment in VLS.

Interactions between vulvovaginal disorders and urinary disorders: The case for an integrated view of the pelvis.

Lower urinary tract symptomatology is often difficult to categorize if history and investigation focus only on the urinary tract. Disease and dysfunction in organs more posteriorly can often cause or influence such bladder and urethral symptoms. Vulvovaginal skin diseases are an important but often missed influence on lower urinary tract symptomatology.

Use of narrowband ultraviolet B (NBUVB) in paediatric psoriasis: A systematic literature review and meta-analysis.

BACKGROUND/ OBJECTIVES: Phototherapy is used commonly in adult psoriasis; however, there is little data on its use in the paediatric population. We performed a systematic review and meta-analysis on current available literature to review the safety and efficacy of NBUVB in paediatric psoriasis. METHODS: A systematic review and meta-analysis according to PRISMA guidelines. Meta-analysis of proportions was used to obtain efficacy, and meta-regression was used to explore the impact of treatment variables on efficacy. RESULTS: We identified 10 prospective and retrospective studies for inclusion. From pooled data, we determined the efficacy of NBUVB in paediatric psoriasis to be 80% (CI 70%-88%). There was a positive association between the number of treatments and efficacy of NBUVB. There was no association between the cumulative dose and efficacy. Erythema was the most commonly described side effect. There were no studies of long-term safety of NBUVB in paediatric psoriasis. Limitations included that studies had small patient numbers and were observational in nature. CONCLUSIONS: NBUVB is effective in the treatment of paediatric psoriasis with a good short-term safety profile. More studies are required to assess the influence of variables on efficacy and to elucidate the long-term safety profile of NBUVB in paediatric psoriasis.