Lichen Planus Pemphigoides Research Articles

51578 Lichen planus pemphigoides: a disease with a nonexistent ICD 10 code of its own – a series of 12 Indian patients

51578 Lichen planus pemphigoides: a disease with a nonexistent ICD 10 code of its own – a series of 12 Indian patients

54707 Lichen planus pemphigoides – A retrospective single-center cohort over two decades and comparison of idiopathic vs. drug-induced cases

54707 Lichen planus pemphigoides – A retrospective single-center cohort over two decades and comparison of idiopathic vs. drug-induced cases

A rare case of lichen planus pemphigoides induced by metformin

Abstract: Metformin is an oral antidiabetic drug of the class biguanides. It can rarely cause dermatological adverse drug reactions (ADRs), of which, lichen planus pemphigoides is an extremely rare ADR. This case report emphasizes the chance of potential immune reaction with metformin and the importance of early diagnosis and treatment of the ADR.

Bullous pemphigoid and lichen planus pemphigoides in monozygotic twins with a long-standing history of psoriasis.

In the medical literature, occurrence of pemphigoid diseases in twins has rarely been reported in the literature. We report identical twins with a long-standing history of psoriasis who developed in their third decades bullous pemphigoid (BP) and lichen planus pemphigoides (LPP), respectively.

Lichen Planus Pemphigoides Induced by Camrelizumab in Combination With Lenvatinib.

Lichen Planus Pemphigoides Induced by Camrelizumab in Combination With Lenvatinib.

Patterns of Periodic Acid–Schiff Staining in Subepidermal Bullous Disorders with Emphasis on Bullous Pemphigoid

Abstract Background: Direct immunofluorescence (DIF) including salt split skin technique is quintessential in the diagnosis of subepidermal bullous disorders. These techniques are expensive and require technical expertise, limiting their diagnostic utility. The property of periodic-acid–Schiff to stain the basement membrane and the patterns produced by it was observed in a cohort of subepidermal bullous disorders, to see if it offers an alternative cost-effective testing method. Materials and Methods: This was a retrospective, observational study conducted in the department of general pathology, in a tertiary care medical center, over a period of 4 years. Only those cases for which paraffin sections, DIF and direct salt split skin immunofluorescence technique were available were included. The levels of anti-bullous pemphigoid (BP) antibodies were collected where available. Results: Of the 21 cases analyzed, 15 were BP, two each of epidermolysis bullosa acquisita (EBA) and lichen planus pemphigoides (LPP), one each of porphyria cutanea tarda (PCT), and bullous systemic lupus erythematosus (SLE). All the cases of BP, LPP and EBA showed a floor pattern of staining with periodic acid–Schiff (PAS) stain. Roof pattern of staining was observed in bullous SLE and PCT. PAS was found to be useful in diagnosing BP, especially in conjunction with an eosinophil rich bulla and anti-BP antibodies. Conclusion: We found that PAS stain could be an adjunct to hematoxylin and eosin stain when the diagnosis of BP is in doubt. However, larger sample size is needed to study its utility in other subepidermal bullous disorders.

Lichen planus pemphigoides erupting after COVID‐19 vaccination and infection, successfully treated with methotrexate

AbstractWe report a case of lichen planus pemphigoides (LPP) in a 36‐year‐old woman who presented bullous skin eruptions following both COVID‐19 infection and vaccination. The patient had a history of biopsy‐confirmed lichen planus since 2013 and had been previously investigated for suspected dermatitis herpetiformis since 2014, with inconclusive biopsy results. On two separate occasions, following her COVID‐19 vaccination and infection, she suffered the eruption of similar bullous lesions. A final diagnosis of LPP was established, believed to have been triggered by both the vaccination and the COVID‐19 infection. Treatment with prednisolone offered temporary relief, but sustained remission was achieved with methotrexate. This is, to our knowledge, the first documented case of LPP post‐COVID‐19 vaccination or infection.

Rare variants of pemphigoid diseases

Pemphigoid diseases comprise aheterogeneous group of subepidermal autoimmune blistering dermatoses characterized by autoantibodies against structural proteins of the dermal-epidermal junction. Recent decades have witnessed asignificant surge in the incidence of these diseases, which, in addition to general aging of the population, can be attributed to the availability of precise diagnostic methods and improved knowledge of the clinical and immunopathological spectrum. While bullous pemphigoid, mucous membrane pemphigoid, and linear IgA disease account for most pemphigoid disorders, less frequent, presumably underdiagnosed variants are increasingly becoming relevant for clinicians. These include epidermolysis bullosa acquisita, anti-p200 pemphigoid, pemphigoid gestationis, lichen planus pemphigoides, and recently defined entities such as IgM pemphigoid and Orf-induced pemphigoid. Accurate characterization and differentiation of these subtypes are not only of diagnostic relevance but may also be associated with therapeutic and prognostic implications for affected individuals. Due to the rarity of these diseases, no controlled prospective clinical trials currently exist, making their diagnosis and therapy challenging.

Involvement of palms and soles in patients with autoimmune bullous diseases: a comparative analysis of a diagnostically relevant localization.

The involvement of palms and soles is variable among disease entities belonging to autoimmune bullous diseases (AIBD). We present our own clinical-laboratory experience concerning presentations of skin lesions on palms and soles in the pemphigus diseases group, pemphigoid diseases group, epidermolysis bullosa acquisita (EBA), and lichen planus pemphigoides (LPP) and discuss the pertinent literature. Lesions on palms and soles were assessed retrospectively on the basis of just photographic archives from the beginning of 2014 to March 2023. We comparatively evaluated 462 Slavic patients with AIBD. Palmoplantar involvement was observed in only 21 patients with AIBD (12 females and 9 males). There was no statistically significant difference between palmoplantar involvement in the pemphigus diseases group compared to the pemphigoid diseases group and no statistically significant difference between the pemphigus diseases group compared to the subepithelial AIBD. Nevertheless, particularly in LPP and EBA, and occasionally in pemphigus diseases and pemphigoid diseases groups of AIBD, localization on palms and soles may be diagnostically important at the clinical level.

Desquamative gingivitis treatment with topical tacrolimus applied to a custom tray: an open trial regarding its efficacy on patients’ symptoms

To evaluate the efficacy of topical tacrolimus offered on a custom tray to treat desquamative gingivitis (DG). Eighteen patients with symptomatic DG related to oral lichen planus (OLP) or mucous membrane pemphigoid (MMP) were selected, of which 13 completed the study. Periodontal treatment was followed by the fabrication of a custom silicone tray to apply a tacrolimus gel formulation (0.1%). Clinical evaluation (complaint of pain and burning - visual analog scale from 0 to 10; and the presence of erythema, desquamation, vesicle/blister, erosion, ulcer, and bleeding) was performed by the same examiner on day 1, and every 15 days for 90 days. Total remission was found in 4 patients (30.76%). Partial remission was found in 69.24% of the patients, classified with an excellent (30.76%), good (30.76%), and regular (7.69%) recovery, respectively. There was a reduction of about 60% in pain and 65% in burning sensation complaints. Wilcoxon test revealed significant differences between pre- and post-treatment pain and burning sensation symptoms (P < .01). Topical application of 0.1% tacrolimus gel was effective in the treatment of DG in controlling pain and burning sensation, leading to the clinical remission of gingival lesions in patients with OLP and MMP.

Autoimmune bullous dermatoses in cancer patients treated by immunotherapy: a literature review and Italian multicentric experience.

Cutaneous immune-related adverse events are frequently associated with immune checkpoint inhibitors (ICIs) administration in cancer patients. In fact, these monoclonal antibodies bind the cytotoxic T-lymphocyte antigen-4 and programmed cell death-1/ligand 1 leading to a non-specific activation of the immune system against both tumoral cells and self-antigens. The skin is the most frequently affected organ system appearing involved especially by inflammatory manifestations such as maculopapular, lichenoid, psoriatic, and eczematous eruptions. Although less common, ICI-induced autoimmune blistering diseases have also been reported, with an estimated overall incidence of less than 5%. Bullous pemphigoid-like eruption is the predominant phenotype, while lichen planus pemphigoides, pemphigus vulgaris, and mucous membrane pemphigoid have been described anecdotally. Overall, they have a wide range of clinical presentations and often overlap with each other leading to a delayed diagnosis. Achieving adequate control of skin toxicity in these cases often requires immunosuppressive systemic therapies and/or interruption of ICI treatment, presenting a therapeutic challenge in the context of cancer management. In this study, we present a case series from Italy based on a multicenter, retrospective, observational study, which included 45 patients treated with ICIs who developed ICI-induced bullous pemphigoid. In addition, we performed a comprehensive review to identify the cases reported in the literature on ICI-induced autoimmune bullous diseases. Several theories seeking their underlying pathogenesis have been reported and this work aims to better understand what is known so far on this issue.

Pruritus Is Associated with an Increased Risk for the Diagnosis of Autoimmune Skin Blistering Diseases: A Propensity-Matched Global Study.

Autoimmune bullous skin diseases (AIBDs), such as bullous pemphigoid (BP) and pemphigus, are characterized and caused by autoantibodies targeting structural proteins. In BP, clinical experience and recent systematic evaluation identified pruritus to be common and an important cause of impaired quality of life. Furthermore, chronic pruritus may be the sole clinical symptom of BP. In pemphigus, a retrospective study recently documented a high prevalence of pruritus. The temporal relation between pruritus and BP/pemphigus are, however, unknown. Likewise, the presence of pruritus in AIBDs other than BP and pemphigus is unknown. To address this, we performed propensity-matched retrospective cohort studies using TriNetX, providing real-world patient data to (i) assess the risk to develop AIBDs following the diagnosis of pruritus and (ii) vice versa. We assessed this in eight AIBDs: BP, mucous membrane pemphigoid (MMP), epidermolysis bullosa acquisita, dermatitis herpetiformis, lichen planus pemphigoides (LPP), pemphigus vulgaris, pemphigus foliaceous, and paraneoplastic pemphigus (PNP). For all AIBDs, pruritus was associated with an increased risk for the subsequent diagnosis of each of the eight investigated AIBDs in 1,717,744 cases (pruritus) compared with 1,717,744 controls. The observed hazard ratios ranged from 4.2 (CI 3.2-5.5; p < 0.0001) in MMP to 28.7 (CI 3.9-211.3; p < 0.0001) in LPP. Results were confirmed in two subgroup analyses. When restricting the observation time to 6 months after pruritus onset, most HRs noticeably increased, e.g., from 6.9 (CI 6.2-7.9; p < 0.0001) to 23.3 (CI 17.0-31.8; p < 0.0001) in BP. Moreover, pruritus frequently developed following the diagnosis of any of the eight AIBDs, except for PNP. Thus, all AIBDs should be considered as differential diagnosis in patients with chronic pruritus.

Oral lichen planus pemphigoid : a case report of a rare disease treated by topical Tacrolimus mouthwash and Hydroxychloroquine

Introduction: Oral Lichen Planus Pemphigoid (LPP) is the rare association of lichen planus and Mucous Membrane Pemphigoid (MMP) whose therapeutic lines are poorly defined. Bilateral and symmetrical reticulated white lesions characteristic of lichen planus (LP) most often precede the painful post-bullous ulcerations of pemphigoid (P). The oral LPP is very rare and the treatment is not codified. This report described a rare case of oral LPP, successfully treated with an original combo of topical Tacrolimus and hydroxychloroquine. Observation: A 72 yr old patient was referred to the unit of the oral mucosa pathology and oro-facial pain, of the oral surgery service of Bordeaux hospital. He has painful mouth ulcerations accompanied by reticulated and symmetrical white lesions. A biopsy to perform an anatomopathological examination and a direct immunofluorescence founds the anatomopathological characteristics of LP and P.As first line of treatment, local and general corticosteroid therapy was undertaken to reduce inflammation. The result was not totally satisfactory. As second line of treatment topical Tacrolimus and hydroxychloroquine made it possible to reduce drastically the patient's erosive and ulcerated lesions. Conclusion: The combination of topical tacrolimus and HCQ should be considerated as second line of treatment for LPP resistant to corticotherapy.

Methotrexate in Lichen Planus Pemphigoides - A Case Report and Review of the Literature.

Methotrexate in Lichen Planus Pemphigoides - A Case Report and Review of the Literature.

Oral lichen planus and related lesions. What should we accept based on the available evidence?

Recent new terminologies have been proposed for lesions in the sphere of oral lichen planus (OLP) that theoretically present unique aetiological, clinical, prognostic or management characteristics different from those of the so-called typical forms of OLP. We aimed to critically analyse what concepts and terminologies related to OLP should we accept based on the available evidence. A review of the literature was carried out in order to critically analyse the concepts and terminologies related to OLP. New concepts and terminologies include oral lichenoid lesions; contact lichenoid reactions, drug lichenoid reactions or those in the context of graft-versus-host disease; chronic ulcerative stomatitis; lichen planus pemphigoid; and some lesions that are difficult to categorise, such as OLP with features of proliferative verrucous leukoplakia and lichenoid lesions of the upper labial mucosa. A multidisciplinary, multicontinent working group has recently published a guideline with recommendations for modifying definitions and terminologies associated with a disease, among which a reasoned, evidence-based justification for the proposed change is considered essential. An in-depth analysis of the newly proposed terms for OLP-related lesions shows that many of them are not justified. In this paper, we set out our position on the basis of the existing evidence on the appropriateness of the use of these new terms.

502 Analysis of checkpoint inhibitor therapy-associated pemphigoid in a series of five Bulgarian patients

Programmed cell death 1 (PD-1) inhibitors used as target anti-cancer immunotherapy have been largely reported for their cutaneous immune-related adverse events (irAEs) ranging from xeroderma or pruritus to atopic dermatitis, psoriasis, vitiligo, etc. Autoimmune blistering diseases (AIBD) from the pemphigoid spectrum, namely bullous pemphigoid (BP) and rarely lichen planus pemphigoides (LPP) have also been associated with checkpoint inhibitors (CI). There is a growing interest in investigating their causal relation, characteristics, and treatment response.

Dermatopathology of COVID-19 infection and vaccination.

BackgroundMany peculiar skin changes have been described in relation to both coronavirus disease 2019 (COVID-19) infection and vaccination.ObjectiveThis paper provides an overview of these dermatologic manifestations, focusing on their dermatopathological appearances.ResultsMost COVID-19 patients develop variegated maculopapular eruptions with a combination of histological patterns commonly including keratinocyte apoptosis and eosinophilia. Urticaria-like lesions often show a combination of spongiotic and lichenoid patterns and are more frequent in individuals with severe disease. Vesicular lesions can be disseminated; in some cases, they become pustular and in others show dyskeratosis and a peculiar form of ballooning. Some patients develop vesicular Grover disease on the trunk. Young patients with a strong immunological response can eliminate the virus easily but may develop chilblains as a consequence of the high interferon response. Conversely, older individuals with immunosenescence and a tendency toward hypercoagulability can present livedoid and ischemic areas. Regarding COVID-19 vaccination, hypersensitivity reactions are most frequent, including “COVID-arm.” Nonetheless, a combination of local and systemic cutaneous manifestations (reactogenicity) is commonly seen. Histopathologically, lichenoid and spongiotic changes and a variable number of eosinophils are typical of rashes characterized by papules and plaques. Other dermatological side effects of COVID-19 vaccination include lesions mimicking well-defined dermatoses such as lichen planus or bullous pemphigoid and triggering of collagenous diseases.ConclusionWell-characterized skin manifestations of coronavirus disease 2019 (COVID-19) include chilblains, livedo necrotic lesions, vesicular eruptions, urticarial lesions, and maculopapular eruptions. Hypersensitivity reactions are common after SARS-CoV‑2 mRNA vaccination. Vaccine reactions may also mimic other dermatosis such as bullous pemphigoid or lichen planus, stimulate herpes reactivation, or trigger the development of autoimmune diseases.

Desquamative gingivitis as a sole presentation in mucous membrane pemphigoid: A rare case report with a brief review of literature

Desquamative gingivitis refers to an erythematous, ulcerated, or atrophic appearance of the gingiva. This clinical appearance is common to a number of pathologies, most frequently accounting for mucocutaneous autoimmune entities (Oral lichen planus and Mucous membrane pemphigoid). The oral physician must be well versed with its varied clinical presentations, and a prompt diagnosis is essential for early remission from this debilitating state.

Treatment of lichen planus pemphigoides with mesalamine: novel use of this drug

Treatment of lichen planus pemphigoides with mesalamine: novel use of this drug

CLINICOPATHOLOGIC FEATURES OF ORAL CAVITY VESICULOBULLOUS DISEASES SEEN IN THE UF ORAL MEDICINE AND DERMATOLOGY CLINICS

<h3>Introduction</h3> Vesiculobullous disorders encompass several rare conditions with varying pathology and morbidity, but similar signs and symptoms. <h3>Materials and Methods</h3> With IRB approval, the record systems of the UF Oral Medicine and UF Dermatology Clinics were searched for cases diagnosed between 2011 and 2020 of the following disorders: pemphigus vulgaris (PV), mucous membrane pemphigoid (MMP), bullous pemphigoid (BP), linear IgA disease, lichen planus pemphigoides, paraneoplastic pemphigus, epidermolysis bullosa acquisita, and angina bullosa hemorrhagica. Exclusion criteria included lack of oral involvement or lack of clinical information and outcome data. Location of lesions within the oral cavity, clinical signs and symptoms, treatment provided, and outcome of treatment were recorded. <h3>Results</h3> A total of 38 cases were included in the study consisting of 20 (52.6%) cases of MMP, 9 (23.7%) cases of PV, 6 (15.8%) cases of BP, and 3 (7.9%) cases of EBA. Twenty-two patients were male (57.9%) and 16 were female (42.1%). Age range was 34 to 92 years, with a mean of 69.7 years. EBA was seen in patients with the highest average age (74.7 years) while patients with PV were the youngest on average (59.8 years). About 90% of MMP cases affected the gingiva, while only one case (16.7%) of BP involved the gingiva. Pain was the most common feature associated with both types of pemphigoid. Ulcerations and erosions were the most common features seen in PV and EBA. All (100%) of EBA cases, 88.9% of PV cases, 66.7% of BP cases, and 25.0% of MMP cases required treatment beyond topical steroids. These therapies included topical tacrolimus, prednisone, methotrexate, rituximab, mycophenolate, and dapsone. PV exhibited the highest percentage (22.2%) of cases refractory to treatment. <h3>Conclusion</h3> Most MMP cases were effectively managed with topical steroids. EBV, PV, and BP may require more potent therapy.