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.

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.

Exploring Remission Dynamics and Prognostic Factors in Lichen Planopilaris: A Retrospective Cohort Study.

INTRODUCTION: Lichen planopilaris (LPP) is a common type of primary cicatricial alopecia. Previous studies focused on the epidemiology, clinical characteristics, and treatment of LPP. A lack of knowledge regarding LPP outcomes and prognostic factors remained. METHODS: To delineate the rate and timing of remission in LPP, as well as the prognostic factors for achieving remission, a retrospective cohort study was conducted. The study included 126 patients, from a single tertiary center, diagnosed with LPP between January 2010 and December 2022, who were followed up for a minimum of 6 months. RESULTS: There were 89 (70.6%) women and 37 (29.4%) men included in this study. The mean age of the patients was 47.92 ± 14.2 years. The mean time from disease onset to diagnosis was 33.85 (±30) months, indicating significant diagnostic delays. The mean duration of follow-up was 34.13 ± 22.7 months. Among the cohort, 43 patients achieved complete remission (CR) during the follow-up period, whereas 83 patients did not. Of the 83 patients who did not achieve CR, 35 partially improved and 48 did not improve or worsened. The median time for achieving CR was 46 ± 18.8 months. Milder disease at presentation and comorbid lichen planus were associated with higher CR rates. CONCLUSION: This study demonstrates significant diagnostic delays that should be addressed as LPP causes irreversible alopecia, suggests disease severity and comorbid lichen planus as potential prognostic factors. Further, it emphasizes the limited efficacy of current treatments and the need for prolonged treatment in patients with LPP to achieve remission.

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.

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.

Hyperpigmented Macules and Patches on the Face: Exogenous Ochronosis or Lichen Planus Pigmentosus?

We present a case of a patient with a 10-year history of blue-black macules and patches on the face and an associated history of skin-lightening cream usage. The skin lightening cream contained hydroquinone, which is often associated with exogenous ochronosis (EO). Interestingly, the biopsy did not show characteristic findings of ochronosis, confusing the final diagnosis, however discontinuing the skin-lightening creams halted the progression of the patient's skin lesions supporting a diagnosis of EO. EO presents as asymptomatic hyperpigmentation after using products containing hydroquinone. This condition is most common in Black populations, likely due to the increased use of skin care products and bleaching cream containing hydroquinone in these populations. Topical hydroquinone is FDA-approved to treat melasma, chloasma, freckles, senile lentigines, and hyperpigmentation and is available by prescription only in the US and Canada. However, with the increased use of skin-lightening creams in certain populations, it is important for dermatologists to accurately recognize the clinical features of exogenous ochronosis to differentiate it from similar dermatoses. An earlier diagnosis can prevent the progression to severe presentations with papules and nodules. We summarize the clinical presentations diagnostic features, and treatment pearls, concluding with a discussion of the differential diagnoses.  J Drugs Dermatol. 2024;23(7):567-568.     doi:10.36849/JDD.8248.

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 Treated with Individualized Homeopathic Medicine, A Case Report.

Context: Lichen planus (LP) is a chronic lichenoid inflammatory disease of the skin, mucosa and appendages. The classic LP symptom is a dense infiltration of inflammatory T cells moving in the upper dermis and arranged in a band-like pattern. Lichen planus has an undetermined aetiology; however, it is known to have immune-mediated pathogenesis. Lichen planus cannot be cured, although treatment can lessen symptoms and shield against further problems. Antihistamines, PUVA (psoralen plus ultraviolet) treatment, retinoic acid, tacrolimus ointment, pimecrolimus cream, as well as corticosteroids are among the most often used therapies. To treat Lichen planus, individualized homeopathic medicine (iHOM) has shown excellent success. Methods: The case was documented at the dermatology OPD (Outpatient Department) of Dr. DY Patil Homoeopathic Medical College and Research Centre. A 32-year-old male patient with lichen planus was treated with individualized homeopathic medicine (iHOM) from March 25, 2021, to August 12, 2021. The results were evaluated at the follow-up visits. An evaluation based on the modified Naranjo criteria was carried out to determine if the alterations were brought on by homeopathic medication. Results: Over a five-month observation period, iHOM medicine produced positive results that physicians may utilize as an additional form of treatment for lichen planus. Conclusion: Individualized homeopathic medicine (iHOM) Nitric acid 30C was prescribed based on the totality of symptoms. Within 5 months, the disease's progression was halted, the itching was controlled, and the lesions flattened.

Remission of refractory esophageal lichen planus induced by tofacitinib.

As of now, there exists no established therapy for ELP. Retinoids, which are standard in treating cutaneous LP, do not exhibit positive effects in ELP. While topical glucocorticosteroids often yield favorable responses in esophageal inflammation, some cases prove recalcitrant or refractory. In such instances, various immunosuppressive therapies have been attempted with variable success.This report details a severe case of ELP that showed resistance to prednisolone, acitretin, alitretinoin, adalimumab, tacrolimus, hydroxychloroquine plus mycophenolate mofetil, and cyclophosphamide. The initiation of the JAK inhibitor tofacitinib induced an impressive clinical, endoscopic, and histological remission. This positive response to a JAK inhibitor is discussed in the context of our evolving understanding of the immune-mediated pathogenesis of this disease.

Recalcitrant multi-variant lichen planus successfully treated with oral baricitinib and topical ruxolitinib cream.

Lichen planus is a chronic auto-inflammatory disease that primarily affects mucocutaneous regions. There are many variants of lichen planus including cutaneous, oral, nail, follicular, and erosive forms. Without any disease-specific treatment options, multi-variant lichen planus can be a challenging disease to manage. We present a 61-year-old woman with multivariant lichen planus that was refractory to numerous systemic and topical therapies. Subsequently, her cutaneous and vulvovaginal lesions improved with the use of oral baricitinib and the erosive oral lesions improved with topical ruxolitinib.

Exclusive extragenital lichen sclerosis in a child presenting in a lichen planus distribution.

Lichen sclerosus (LS) is a chronic inflammatory dermatosis primarily affecting the genitalia, commonly characterized by pearly-white papules and plaques. Although predominantly affecting females, LS can manifest across all age groups, with a bimodal distribution observed in prepubescent girls and postmenopausal women. This case report presents an unusual instance of exclusive extragenital LS in a 10-year-old girl, showcasing hyperpigmented patches and wrinkled plaques resembling lichen planus on her forearms and lower legs. Histopathological analysis confirmed LS, revealing distinctive epidermal changes and lymphocytic infiltrates. The absence of mucosal involvement and unique clinical presentation differentiated this case from typical LS manifestations. Treatment with topical clobetasol propionate demonstrated significant improvement in pruritus. Extragenital LS is infrequent, particularly among children, and its diagnosis necessitates a comprehensive clinicopathological correlation. The reported case contributes valuable insights into this uncommon variant, emphasizing the importance of accurate diagnosis and tailored treatment strategies. Additionally, it highlights the efficacy of high-potency topical corticosteroids in managing this condition.

Lichen Planus of the Lip-Case Series and Review of the Literature.

Background and Objectives: Lichen planus of the lip (LPL) is a chronic inflammatory condition that resembles actinic cheilitis, discoid lupus erythematosus, graft-versus-host disease, and lichenoid reaction to dental materials or drugs. The purpose of this study was to conduct a literature review on lichen planus lip involvement and to report a retrospective observational study that characterises and explores the clinical, histopathological, and evolution of the lesions in a group of patients with unique involvement of LPL. Materials and Methods: Clinical data of patients diagnosed with LPL was retrieved from the medical charts of the patients referred to the Oral Pathology Department of the "Carol Davila" University of Medicine and Pharmacy. A concurrent electronic literature research was carried out using PubMed and Web of Science from 2003 to 2023. Results: Eleven patients diagnosed with unique LPL were analysed (male/female ratio was 1.75, mean age 63.64 years ± 12.52). All patients presented lesions of the lower lip; the clinical forms were atrophic (six cases) and erosive (five cases), and the histopathological exam confirmed the diagnosis. After topical treatment with corticosteroids, most of the patients had complete remission. The literature review revealed 24 studies (sixteen case reports and eight case series) which comprised 84 patients. Isolated lip involvement was reported in 17 studies, and five articles with concomitant oral lichen planus, while two articles did not mention this criterion. Conclusions: Our study brings new data on isolated lichen planus of the lip that primarily affects the lower lip with predominance in male patients. It was reported worldwide in patients between 22 and 75 years old. Topical corticosteroids were the main treatment prescribed and they usually brought remission of the lesions. Lichen planus of the lip is a challenging diagnosis for oral health practitioner providers as well as for dermatologists.

Lichen planus esophagitis.

PURPOSE OF REVIEW: Dysphagia is one of the most common reasons for patients' visits to a gastroenterologist. Esophageal lichen planus (ELP) has historically been felt to be a rare disease, when in fact it is often misdiagnosed and unrecognized. Often first diagnosed as an unusual esophagitis, all gastroenterologists will see ELP in their practice, and need to be able to recognize this condition. RECENT FINDINGS: Although there is still a relative paucity of data on this condition, this article will update the typical presenting symptoms, endoscopic findings, and ways to differentiate ELP from other inflammatory mucosal diseases. There is still no standardized treatment algorithm, but we will also present the most recent treatment approaches. SUMMARY: It is critical that physicians maintain an increased awareness of ELP and have a high clinical suspicion in the appropriate patients. While management remains challenging, it is important to treat both the inflammatory and stricturing components of the disease. A multidisciplinary approach is also often required, utilizing dermatologists, gynecologists, and dentists who are familiar with managing patients with LP.

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.

Lichen Planopilaris Pemphigoides: A Novel Bullous Dermatosis Due to Programmed Cell Death Protein-1 Inhibitor Therapy.

ABSTRACT: Lichen planus pemphigoides (LPPemph), apart from bullous pemphigoid, is a rare bullous dermatosis that can be induced by programmed cell death protein-1 (PD-1)/PD ligand 1 (PD-L1) inhibitors. The primary location of PD-1/PD-L1 inhibitor-induced LPPemph has previously only been reported at the nonfollicular dermal-epidermal junction. We present a case of nivolumab-induced LPPemph with an intense perifollicular lichenoid reaction, prominent multifocal perifollicular clefting, which in addition, was also accompanied by linear IgG and C3 immunofluorescence deposits along the dermal-epidermal junction as well as demonstrating a perifollicular pattern. Intriguingly, the serological study of BP180 and BP230 antibodies was negative, suggesting the presence of additional novel antibodies, which primarily favor hair follicles and may contribute to the pathogenesis. Therefore, we consider this entity a novel variant of PD-1/PD-L1 inhibitor-induced bullous dermatosis. To the best of our knowledge, this is the first report that highlights perifollicular bullae accompanied by immunofluorescence findings in a PD-1/PD-L1 inhibitor-induced lesion. We propose a new immunotherapy associated entity, lichen planopilaris pemphigoides, and emphasize the significance of perifollicular changes in the pathogenesis.

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.

Acute Onset Linear Lichen Planus Pigmentosus of the Forehead: A Case Series.

Linear lichen planus pigmentosus (LPP) of the face is a rare variant of lichen planus, with only a few cases published in the literature.1 It is an inflammatory condition with unknown etiology, characterized by blue-gray hyperpigmented macules, and tends to affect sun-exposed areas of the head and neck.1-4 The pathophysiology of linear lichen pigmentosus is poorly understood, though it is postulated to be caused by T-lymphocyte autoimmunity against keratinocytes.5-7 LPP more frequently affects middle age woman and skin phototypes III-VI.1,3 Treatment for linear LPP is difficult and there is no established first-line therapy; however, tacrolimus ointment, topical corticosteroids, and various systemic agents have shown to be effective in improving the appearance.3,8,9 Prior reports have characterized linear LPP that follows the lines of Blashko as more commonly affecting the trunk.1 We present three cases of linear lichen planus pigmentosus (LPP) of the forehead, a unique novel presentation of linear LPP of the face. One of our cases also provides supporting evidence for tacrolimus to be used as a preferred therapy to treat linear LPP of the face; however, more research is needed to support this claim. To our knowledge, this case series is the largest case series of linear lichen planus pigmentosus (LPP) of the forehead to be reported. J Drugs Dermatol. 2023;22(1):94-97. doi:10.36849/JDD.7200.

Graham-Little-Piccardi-Lasseur Syndrome: A Case Report.

Graham-Little Piccardi-Lasseur syndrome is a rare dermatosis that affects the hair follicles throughout the body and often presents with a progressive cicatricial alopecia of the scalp that is unresponsive to medical therapy. While treatment options are limited, prompt recognition through a careful physical exam aided by dermoscopy can facilitate early intervention. Here we present a patient with GLPLS, discuss pertinent morphologic and dermoscopic findings, and review the current literature. J Drugs Dermatol. 2022;22(2):210-216. doi:10.36849/JDD.6926.

Pediatric lichen planus: A single-center retrospective review of 26 patients with follow up.

BACKGROUND/OBJECTIVES: Pediatric lichen planus (LP) is rare with variable prevalence and atypical presentations compared to adults. Data on LP are lacking for the pediatric population in the United States. We present demographics, presentations, and treatments for a pediatric LP cohort. METHODS: We reviewed 26 patients diagnosed with LP at 20 years or younger. Treatment responses were defined as no response, partial response, and complete response. RESULTS: Demographics included 54% females and median diagnosis age of 16 years (range 6-20). Most patients presented with cutaneous LP (65%), with fewer having associated oral (23%), nail (7.7%), or genital (3.8%) involvement. Some had cutaneous-only LP (38%) or strictly mucosal LP (oral-only 19% and genital-only 15%). LP lesions were pruritic (50%), painful (19%), and/or asymptomatic (35%). Complete/partial responses occurred with medium-potency topical corticosteroids in cutaneous (n = 7; 64%), oral (n = 3; 75%), and genital LP (n = 3; 100%), with high/ultra-high potency topical corticosteroids in oral LP (n = 6; 86%), and with topical calcineurin inhibitors in genital LP (n = 2; 100%). Side effects were clobetasol-related oral candidiasis and biopsy-related penile depressed scar. Most patients with available follow-up achieved remission (n = 17; 81%). CONCLUSIONS: Pediatric LP usually presents in adolescence with cutaneous involvement and is symptomatic. However, patients frequently can have oral, genital, or nail lesions or may be asymptomatic, so they need thorough examinations and follow-up. Long-term remission is common due to treatment or natural disease course. Medium-potency corticosteroids are recommended for cutaneous, oral, and genital LP. Various other local and systemic therapies exist with successful treatment responses.