Lichen planus pemphigoides with predominant mucous membrane involvement: a series of 12 patients and a literature review.

Background: Lichen planus pemphigoides (LPP), an association between lichen planus and bullous pemphigoid lesions, is a rare subepithelial autoimmune bullous disease. Mucous membrane involvement has been reported previously; however, it has never been specifically studied. Methods: We report on 12 cases of LPP with predominant or exclusive mucous membrane involvement. The diagnosis of LPP was based on the presence of lichenoid infiltrates in histology and immune deposits in the basement membrane zone in direct immunofluorescence and/or immunoelectron microscopy. Our systematic review of the literature, performed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, highlights the clinical and immunological characteristics of LPP, with or without mucous membrane involvement. Results: Corticosteroids are the most frequently used treatment, with better outcomes in LPP with skin involvement alone than in that with mucous membrane involvement. Our results suggest that immunomodulators represent an alternative first-line treatment for patients with predominant mucous membrane involvement.

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.

Clinical Characterization and Treatment Response of Folliculitis Decalvans Lichen Planopilaris Phenotypic Spectrum: A Unicentre Retrospective Series of 31 Patients.

Folliculitis decalvans and lichen planopilaris phenotypic spectrum has been described as a form of cicatricial alopecia. The aim of this study is to describe the clinical and trichoscopic features and therapeutic management of this condition in a series of patients. A retrospective observational unicentre study was designed including patients with folliculitis decalvans and lichen planopilaris phenotypic spectrum confirmed with biopsy. A total of 31 patients (20 females) were included. The most common presentation was an isolated plaque of alopecia (61.3%) in the vertex. Trichoscopy revealed hair tufting with perifollicular white scaling in all cases. The duration of the condition was the only factor associated with large plaques (grade III) of alopecia (p = 0.026). The mean time to transition from the classic presentation of folliculitis decalvans to folliculitis decalvans and lichen planopilaris phenotypic spectrum was 5.2 years. The most frequently used treatments were topical steroids (80.6%), intralesional steroids (64.5%) and topical antibiotics (32.3%). Nine clinical relapses were detected after a mean time of 18 months (range 12-23 months). Folliculitis decalvans and lichen planopilaris phenotypic spectrum is an infrequent, but probably underdiagnosed, cicatricial alopecia. Treatment with anti-inflammatory drugs used for lichen planopilaris may be an adequate approach.

[Hydroxychloroquine-not always a harmless drug for off label use in dermatology]. / Hydroxychloroquin ­ nicht immer ein harmloses Medikament für den Off-label-Gebrauch in der Dermatologie.

Hydroxychloroquine is used for treatment of inflammatory diseases. It is considered to have few adverse effects. We report on a woman who developed a severe skin rash after intake of hydroxychloroquine, which she received for treatment of her lichen planopilaris. Based on the clinical, laboratory and histological findings the diagnosis of a drug reaction with eosinophilia and systemic symptoms (DRESS)-like drug reaction was established. Our case illustrates that hydroxychloroquine can lead to severe adverse effects in rare cases and that patients receiving this drug must be thoroughly informed.

Photodynamic therapy for refractory erosive oral lichen planus: a case series study.

Oral lichen planus is a chronic inflammatory disease that occurs on the oral mucosa and is an oral potentially malignant disease. Treatment of oral lichen planus aims to promote healing of the erosion, relieve pain, reduce recurrence of the erosion, and prevent canceration. Corticosteroids are the first line of treatment for oral lichen planus. Refractory oral lichen planus is a clinical classification of oral lichen planus that has not responded to corticosteroid treatment for more than 2 months. Topical 5-aminolevulinic acid-mediated photodynamic therapy is an innovative and effective treatment for potentially malignant oral diseases and has been reported as an alternative treatment to conventional therapies for oral lichen planus. On this basis, we report 3 cases of refractory erosive oral lichen planus in which persistent erosive lesions healed after topical application of 5-aminolevulinic acid-mediated photodynamic therapy without any adverse effects or any signs of recurrence. Topical 5-aminolevulinic acid-mediated photodynamic therapy will become an effective clinical treatment for refractory erosive oral lichen planus.

Ultrasound Features of Nail Lichen Planus.

OBJECTIVES: To describe the ultrasonographic features of nail lichen planus (NLP), which, so far, have not been reported in the literature. METHODS: A retrospective single-center study of NLP patients' color Doppler ultrasound examinations was performed between March 2014 and January 2023. Inclusion criteria were patients ≥15 years with a confirmed clinical diagnosis of NLP in sequential order. Exclusion criteria included concomitant systemic or local cutaneous or nail diseases and systemic or local nail treatments before the ultrasound examination. The ultrasound protocol included the examination of all fingernails or toenails following the reported protocol for dermatologic ultrasound examinations at high and ultra-high frequencies. Patient demographics and ultrasound features of the nail bed, periungual region, nail plate, and regional vascularity were registered and analyzed statistically. RESULTS: A total of 36 patients met the criteria. All cases presented thickened and decreased echogenicity of the nail bed. A hypoechoic halo surrounding the origin of the nail plate was present in 78% of cases. Exactly 58% of patients presented decreased echogenicity of the proximal periungual dermis, and 86% showed thickening of the periungual dermis. Hypervascularity of the nail bed was seen in 94% of cases. The mean maximum thickness of vessels, the peak systolic velocity of the arterial vessels, and other blood flow findings are provided. CONCLUSION: Ultrasonography can support the diagnosis of NLP, which benefits the non-invasive discrimination of nail conditions and can avoid the potential permanent scars derived from nail biopsies. Furthermore, ultrasound may be a powerful tool to monitor this disease's treatment.

Lichen planus pemphigoides induced by anti-PD-1 antibody: A case only involved in oral mucosa with excellent topical treatment efficiency.

Lichen planus pemphigoides (LPP) is a rare autoimmune subepidermal disease that can occur in patients receiving immune checkpoint inhibitors. Its clinical manifestations are combined with the characteristics of lichen planus with bullous pemphigoid that can occur on either skin or oral mucosa. It should be noted that oral LPP is very rare. Here, we report a novel case of oral LPP induced by an anti-PD-1 agent. The patient presented with typical clinical features in oral mucosa, and the diagnosis was based on histopathology and immunological studies. Given that the patient was receiving an anti-PD-1 agent, topical therapy was chosen, and a nice therapeutic effect was obtained. No significant recurrence was observed after a 2-year follow-up. A good and stable therapeutic effect achieved by rapid and local symptomatic medication suggests that accurate and sensitive diagnosis is necessary.

A Systematic Review and Bayesian Network Meta-Analysis of Medical Therapies for Lichen Planopilaris.

BACKGROUND: Lichen planopilaris (LPP) is a primary chronic lymphocytic cutaneous disorder that selectively destroys the hair follicles, resulting in scarring alopecia. Unfortunately, current available treatments are not fully effective to stop hair loss, and the level of evidence for medical interventions is weak. OBJECTIVES: The present article aimed to determine the efficacy of the different medical interventions in LPP through a network meta-analysis (NMA). METHODS: A systematic review and meta-analysis were performed including randomized trials that report the outcomes of lichen planopilaris activity index (LPPAI). These articles were pooled and a NMA was conducted. RESULTS: A total of seven studies were identified and included in meta-analysis, comprising 251 LPP patients. The NMA showed the mean difference in LLPAI was significantly superior with the combination of clobetasol plus N-acetylcysteine (mean difference: -2.0, 95% CI = -3.43 to -0.51) and the combination of clobetasol plus pentoxifylline (mean difference: -1.62, 95% CI = -3.0 to -0.25) compared to the treatment of reference (clobetasol). The NMA showed cyclosporine (mean difference: 2.05 95% CI = 0.68-3.49), methotrexate (mean difference: 1.95 95% CI = 1.23-3.17), the combination of methotrexate plus prednisolone (mean difference: 1.56 95% CI = 0.25-2.96) were significantly worse than hydroxychloroquine according to the differences in LLPAI. CONCLUSION: This work is the first NMA in LPP and hence, it can be helpful in serving as an initial step toward better evidence-based decisions in the treatment of this challenging condition. We propose a triple-combined approach consisting of topical clobetasol, hydroxychloroquine, and N-acetylcysteine as resulted in the most effective approach. Considering the poor outcomes observed with pioglitazone, mycophenolate mofetil, and cyclosporine, it is advisable to contemplate the use of these medications in patients who have not responded adequately to more efficacious alternatives.

Isolated corneal involvement by Lichen Planus - multi-modal evaluation.

PURPOSE: To describe a case of ocular Lichen Planus, successfully managed using a multimodal evaulation, including Anterior Segment OCT (AS-OCT). OBSERVATIONS: A female patient in her forties with a history of cutaneous Lichen Planus presents with blurred vision and burning eye sensation. Anterior segment evaluation revealed bilateral punctate keratitis, stromal haze and subepithelial pigmented dots. AS-OCT was pivotal for diagnosis, showing anterior stromal hyperreflective dots. A diagnosis of ocular Lichen Planus was estabilished and the patient was treated with topical hydrocortisone, with complete subsidence of the complaints. CONCLUSIONS AND IMPORTANCE: Ocular Lichen Planus can present as isolated corneal involvement independent from severe cicatrizing conjunctivitis. Appropriate and timely treatment can prevent irreversible ocular surface disease. Ophthalmologists should be aware of Lichenoid Tissue Reaction (LTR) disorders, especially in patients with relentless blepharitis and/or ocular surface disease.

Esophageal Lichen Planus: A Descriptive Multicenter Report.

GOALS: To better understand the characteristics, treatment approaches, and outcomes of patients with esophageal lichen planus (ELP). BACKGROUND: ELP is a rare, often unrecognized and misdiagnosed disorder. Data on this unique patient population are currently limited to small, single-center series. STUDY: A multicenter, retrospective descriptive study was conducted of adults diagnosed with ELP over a 5-year period, between January 1, 2015, and October 10, 2020, from 7 centers across the United States. RESULTS: Seventy-eight patients (average age 65 y, 86% female, 90% Caucasian) were included. Over half had at least 1 extraesophageal manifestation. Esophageal strictures (54%) and abnormal mucosa (50%) were frequent endoscopic findings, with the proximal esophagus the most common site of stricture. Approximately 20% had normal endoscopic findings. Topical steroids (64%) and/or proton pump inhibitors (74%) dominated management; endoscopic response favored steroids (43% vs. 29% respectively). Almost half of the patients required switching treatment modalities during the study period. Adjunctive therapies varied significantly between centers. CONCLUSIONS: Given its at times subtle clinical and endoscopic signs, a high index of suspicion and biopsy will improve ELP diagnosis, especially in those with extraesophageal manifestations. Effective therapies are lacking and vary significantly. Prospective investigations into optimal treatment regimens are necessary.

Nail psoriasis and nail lichen planus: Updates on diagnosis and management.

BACKGROUND: Inflammatory diseases of the nail, including nail psoriasis and nail lichen planus, are associated with significant disease burden and have a negative impact on quality of life. Diagnosis is often delayed, especially when patients present without cutaneous findings. Therefore, recognizing clinical signs and symptoms of inflammatory nail diseases, and initiating timely and appropriate treatment, is of utmost importance. OBJECTIVE: We review recent studies on diagnostic techniques, discuss severity grading and scoring systems, and describe consensus treatment recommendations for nail psoriasis and nail lichen planus. METHODS: An updated literature review was performed using the PubMed database on studies assessing diagnostic techniques or treatment modalities for nail psoriasis and nail lichen planus. RESULTS: Recent studies on diagnostic techniques for inflammatory nail disease have focused on use of dermoscopy, capillaroscopy, and ultrasound modalities. Treatment of these conditions is dichotomized into involvement of few (≤3) or many (>3) nails. Recent psoriatic therapeutics studied for nail outcomes include brodalumab, tildrakizumab, risankizumab, deucravacitinib, and bimekizumab, while emerging treatments for nail lichen planus include JAK inhibitors and intralesional platelet rich plasma injections. CONCLUSIONS: We emphasize the need for increased awareness and expanded management strategies for inflammatory nail diseases to improve patient outcomes.

Janus-kinase inhibitors in dermatology: A review of their use in psoriasis, vitiligo, systemic lupus erythematosus, hidradenitis suppurativa, dermatomyositis, lichen planus, lichen planopilaris, sarcoidosis and graft-versus-host disease.

Recent studies on molecular pathways have elucidated novel therapeutic approaches in inflammatory and autoimmune skin disorders. Specifically, the dysregulation of the Janus kinase signal transducer and activator of transcription (JAK-STAT) cascade plays a central role in the pathogenesis of many skin conditions. JAK inhibitors, with their ability to selectively target immune responses, are potential treatment options. Using the National Library of Medicine, we provide a comprehensive review of the use of United States Food and Drug Administration (FDA)-approved and emerging JAK or tyrosine kinase 2 (TYK2) inhibitors in a wide range of dermatologic conditions, including psoriasis, vitiligo, systemic lupus erythematosus, hidradenitis suppurativa, dermatomyositis, lichen planus, lichen planopilaris, sarcoidosis and graft-versus-host disease. In patients with psoriasis, oral deucravacitinib (TYK2 inhibitor) has been approved as a once-daily therapy with demonstrated superiority and efficacy over apremilast and placebo and tolerable safety profiles. In patients with vitiligo, topical ruxolitinib (JAK1 inhibitor) is approved as a twice-daily treatment for repigmentation. The efficacy of several other JAK inhibitors has also been demonstrated in several clinical trials and case studies for systemic lupus erythematosus, hidradenitis suppurativa, dermatomyositis, lichen planus, lichen planopilaris, sarcoidosis and graft-versus-host disease. Further investigations with long-term clinical trials are necessary to confirm their utility in treatment and safety for these diseases.

Nail lichen planus treatment safety.

AREAS COVERED: Topical therapies for nail lichen planus (clobetasol propionate, topical tacrolimus, bath-PUVA), intralesional treatment (triamcinolone), and systemic treatment (corticosteroids, retinoids, small molecule inhibitors (jak/stat inhibitors)), TNF-alpha inhibitors (etanercept), systemic immunomodulators (oral calcineurin inhibitors, mycophenolate mophetil), and antimalarials (chloroquine), each with unique safety profiles and considerations. Herein, we discuss common and uncommon adverse events, as well as utilization for special populations, including pregnant and pediatric patients.

Successful Treatment of Lichen Planus With Oral Upadacitinib.

Lichen planus is an auto-inflammatory skin disorder marked by intensely pruritic, violaceous papules that commonly affect the extremities of middle-aged adults.1 There are several treatment options available, but alternative therapies to target disease refractory to standard interventions remain necessary. Though they have not been FDA-approved for lichen planus, Janus kinase (JAK) inhibitors have demonstrated significant potential as a therapeutic intervention across an array of dermatoses. Herein, we present a case of refractory, biopsy-proven lichen planus successfully treated with the oral JAK1 inhibitor, upadacitinib. J Drugs Dermatol. 2023;22(10):1058-1060     doi:10.36849/JDD.7272.

A retrospective cohort study of the efficacy of metronidazole in oral lichen planus.

Lichen planus (LP) is an inflammatory dermatosis with mucosal variants, including oral LP (OLP). OLP has a relapsing chronic course and may significantly affect a patient's quality of life. We analysed the efficacy of metronidazole as a treatment for OLP via a retrospective chart review of patients with a diagnosis of OLP seen at our institution over 10 years. Thirty patients with OLP were treated with metronidazole 500 mg twice daily. Most patients (n = 18; 60%) showed improvement with metronidazole, including 10 (33%) who experienced complete resolution of symptoms. Our results were limited by the retrospective nature of the review and the relatively small cohort size. Given the efficacy, mild side-effect profile and the less immunosuppressive nature of metronidazole vs. alternative systemic treatment options, metronidazole is a reasonable option to try in patients with symptomatic OLP who have failed topical treatment options.