Disease severity scoring systems in mucosal lichen planus: A systematic review.

Several scoring systems have been developed to evaluate disease severity in mucosal lichen planus, but only a few have been validated to ensure reproducible and accurate assessment of disease severity. The current systematic review was undertaken to identify clinical severity scoring systems in mucosal lichen planus that have undergone validity or reliability testing and to describe their operating characteristics. We performed a bibliographic search in five databases from their inception to October 2022 for severity scoring systems in mucosal lichen planus that have undergone validity or reliability tests. Quality assessment was conducted using the Joanna Briggs Institute Critical Appraisal tools. We have included 118 studies and identified 11 clinical severity scoring systems for oral lichen planus that have undergone validity or reliability testing. Of these, the most reported were the Thongprasom score, the Oral Disease Severity Score (ODSS) and the REU (Reticular/hyperkeratotic, Erosive/erythematous, Ulcerative) scoring systems. We did not identify clinical scoring systems for extraoral mucosal lichen planus that have undergone validity or reliability testing. The ODSS and REU scoring systems have undergone the highest number of validation attempts and reliability assessments for oral lichen planus respectively. However, we have identified numerous factors that have hampered the universal adoption of a standardised scoring system. There is a need for the development and validation of scoring systems for extraoral mucosal lichen planus.

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.

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.

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.

[Squamous cell carcinoma arising in oral lichen planus : Report of two cases]. / Plattenepithelkarzinom auf dem Boden eines oralen Lichen planus : Bericht zweier Fälle.

Lichen planus is a chronic inflammatory, immunologically mediated mucocutaneous dermatosis. Lichen planus mucosae predominantly affects the oral cavity. Various trigger factors such as bacterial or viral infections, drugs or physical stimuli are discussed in the development of the disease. An association with human papillomavirus infections has also been described, but is not sufficiently proven. Lichen planus mucosae is considered as a premalignant condition, but the malignant transformation rate is low. The risk of malignant transformation is significantly increased in patients with oral lichen planus who smoke, drink alcohol or have hepatitis C. We describe two patients with locally advanced squamous cell carcinoma that developed on a longstanding oral lichen planus. Both cases were successfully treated with radical tumor resection, subsequent tissue reconstruction, and adjuvant radiation/radiochemotherapy.

Assessing the clinical effectiveness of an algorithmic approach for mucosal lichen planus (MLP): A retrospective review.

BACKGROUND: Mucosal lichen planus (MLP) is a therapeutic challenge in need of a new treatment approach because of its debilitating effect on patient's quality of life. OBJECTIVE: We sought to evaluate a standardized treatment plan for patients with MLP. A second objective was to describe the effect of mycophenolate mofetil in this patient population. METHODS: The study retrospectively analyzed 53 patients with MLP treated using a standardized algorithm. The number of MLP lesions, disease activity, and pain at the last visit were compared with baseline scores determined at the initial visit. Results were analyzed using the paired samples t test and confirmed with the Wilcoxon matched pairs signed rank test. RESULTS: The average number of lesions was reduced from 3.77 to 1.67 (P < .001). The average disease activity was reduced from 2.73 to 0.90 (P < .001). Average pain reported decreased from 2.03 to 1.03 (P < .001). LIMITATIONS: This study was a retrospective analysis of a small patient population. There was no universal symptom severity scale used at the time of treatment for some patients. CONCLUSION: The standardized treatment plan reduced symptoms for patients with MLP. Mycophenolate mofetil appears to be a reasonable treatment option for these patients.

Case series of an alternative therapy for generalised lichen planus: Four case studies.

Lichen planus (LP) is an idiopathic, cell-mediated immune disorder, accompanied by itching. Spontaneous remission occurs. Topical and systemic therapies are utilised. Four cases of generalized LP with and without mucosal involvement treated homeopathically are presented. Case 1: A 48-year-old female presented with a 7-month history of generalized itchy rash, which had been diagnosed as LP, treated unsuccessfully with topical steroids and removal of dental fillings. Examination revealed violaceous papules on upper and lower limbs, oral mucosal lesions and an irregular, erythematous, blanching, macular rash on the chest. She received homeopathic Ignatia amara at medication dilution factor (MK) potency, weekly dose and went into remission at 3 months. Patient remains in remission. Case 2: A 65-year-old female presented with a 27-year history of generalized, LP, which had been unresponsive to topical steroids. Examination showed generalized, violaceous papules, with no mucosal involvement. She received homeopathic Aurum metallicum, MK potency, weekly, and went into remission. She relapsed at 8 months after onset of therapy, following a very stressful incident, but gained remission again with Aurum metallicum after 1 month of therapy. She remains in remission. Case 3: A 38-year-old male presented with a 21-year history of generalized LP. Medical history was significant for hepatitis B and asthma. Topical steroid therapy was only partially successful. Examination revealed generalized, violaceous papules, with oral and genital involvement. He received homeopathic Lycopodium at MK potency, weekly, and remitted by 2 months. He remains in remission. Case 4: A 41-year-old male presented with a 12-year history of generalized hypertrophic LP, which had responded partially to topical steroids and ultraviolet A therapy. Medical history was significant for reduced sense of smell. Examination revealed generalized, violaceous, hypertrophic papules and nodules. He received homeopathic Carcinosinum at MK potency and remitted at 6-months. In its long-standing, generalized form, with mucosal involvement, LP may respond to individualized homeopathy. More research may clarify homeopathy's place in LP therapy.

Erosive Lichen Planus.

Lichen planus is an inflammatory mucocutaneous condition with a myriad of clinical manifestations. There are 3 forms of lichen planus that effect the vulva: papulosquamous, hypertrophic, and erosive. Erosive lichen planus can progress to vulvar scaring, vaginal stenosis, and squamous cell carcinoma; these long-term sequelae cause sexual distress, depression, and decreased quality of life for patients. Diagnosis is often delayed because of patient embarrassment or clinician misdiagnosis. Early recognition and treatment is essential to decreasing the morbidity of this condition. Multimodal treatment, along with a multidisciplinary approach, will improve outcomes and further clinical advances in studying this condition.