RESUMO
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.
Assuntos
Doenças Autoimunes , Líquen Plano , Penfigoide Bolhoso , Humanos , Mucosa Bucal/patologia , Líquen Plano/induzido quimicamente , Líquen Plano/tratamento farmacológico , Penfigoide Bolhoso/induzido quimicamente , Penfigoide Bolhoso/tratamento farmacológico , Pele/patologia , Doenças Autoimunes/patologiaRESUMO
BACKGROUND: Programmed cell death protein (PD-1) and programmed death-ligand 1 (PD-L1) inhibition checkpoint blockade leads to various cutaneous adverse reactions, including bullous pemphigoid and lichen-planus-like reactions. However, lichen planus pemphigoides (LPP), manifesting histopathologic features of both lichen planus and bullous pemphigoid, has more rarely been associated with immunotherapy. METHODS: The clinical and histopathologic findings of three patients were examined, and a review of cases of LPP and bullous lichen planus secondary to PD-1 inhibitor therapy was performed. RESULTS: Three patients (two with advanced non-small-cell lung adenocarcinoma and the third with metastatic breast cancer) presented with both lichenoid eruptions and bullae. Biopsy of the lesions revealed lichenoid tissue reactions in all three patients. Together with the histopathologic findings, direct immunofluorescence (DIF) showing linear C3 and IgG deposition and positive enzyme-linked immunosorbent assay (ELISA) showing BP180 positivity supported a diagnosis of LPP in two patients. The third patient in our series also showed confirmatory ELISA testing supporting LPP. CONCLUSIONS: Lichen planus pemphigoides is a distinct cutaneous toxicity to checkpoint inhibitor therapy illustrates a possible pathogenic mechanism and the importance of dermatopathology recognition to render an accurate diagnosis.
Assuntos
Carcinoma Pulmonar de Células não Pequenas , Líquen Plano , Neoplasias Pulmonares , Penfigoide Bolhoso , Proteínas Reguladoras de Apoptose/uso terapêutico , Antígeno B7-H1 , Carcinoma Pulmonar de Células não Pequenas/tratamento farmacológico , Humanos , Inibidores de Checkpoint Imunológico/efeitos adversos , Imunoglobulina G , Líquen Plano/diagnóstico , Neoplasias Pulmonares/tratamento farmacológico , Penfigoide Bolhoso/diagnóstico , Receptor de Morte Celular Programada 1RESUMO
Lichen planus pemphigoides (LPP) and bullous lichen planus (BLP) are rare dermatoses, which are characterised by blisters and lichenoid lesions. Their clinical presentation is heterogenous, displaying overlapping features or mimicking other dermatological diseases. Therefore, diagnosis can often be challenging, requiring a thorough dermatological examination along with distinctive histological and immunopathological characteristics. Lichenoid degeneration of the basal epidermis exposes various antigens of the dermal-epidermal junction in LPP, resulting in the breakdown of immune tolerance, hence, the production of autoantibodies against type XVII collagen. Conversely, no pathogenic autoantibodies are detected in BLP. However, some cases of mucosal lichen planus might display immunopathological features suggestive of autoimmune blistering diseases. Therefore, a better understanding of the pathophysiology of these two distinct dermatoses is imperative. The aim of this review was to provide a summary of the current knowledge on the clinical hallmarks, diagnosis and available therapeutic options in LPP and BLP.
Assuntos
Doenças Autoimunes , Líquen Plano , Penfigoide Bolhoso , Autoanticorpos , Vesícula/diagnóstico , Humanos , Líquen Plano/diagnóstico , Líquen Plano/tratamento farmacológico , Líquen Plano/patologia , Penfigoide Bolhoso/diagnóstico , Penfigoide Bolhoso/tratamento farmacológicoRESUMO
Subepithelial autoimmune blistering dermatoses are a group of rare skin disorders that are characterized by the disruption of the dermal-epidermal junction through the action of autoantibodies. The third article in this continuing medical education series explores the background, epidemiology, clinical features, and diagnostic criteria of each of the major subepithelial autoimmune blistering dermatoses, including bullous pemphigoid, pemphigoid gestationis, lichen planus pemphigoides, mucous membrane pemphigoid, linear IgA bullous dermatosis, and dermatitis herpetiformis.
Assuntos
Doenças Autoimunes/diagnóstico , Líquen Plano/diagnóstico , Penfigoide Gestacional/diagnóstico , Dermatopatias Vesiculobolhosas/diagnóstico , Doenças Autoimunes/epidemiologia , Doenças Autoimunes/imunologia , Doenças Autoimunes/patologia , Derme/imunologia , Derme/patologia , Feminino , Humanos , Líquen Plano/epidemiologia , Líquen Plano/imunologia , Líquen Plano/patologia , Penfigoide Gestacional/epidemiologia , Penfigoide Gestacional/imunologia , Penfigoide Gestacional/patologia , Gravidez , Dermatopatias Vesiculobolhosas/epidemiologia , Dermatopatias Vesiculobolhosas/imunologia , Dermatopatias Vesiculobolhosas/patologiaRESUMO
Over the years, the occurrences of different types of skin disorders arising from radiation sites have been observed and studied. Examples include autoimmune blistering diseases such as pemphigus, pemphigoid, and interface or inflammatory reaction patterns such as lichen planus, lupus erythematosus, and Stevens-Johnson syndrome. The phenomenon whereby a new skin disorder arises from a previously healed or irradiated site is called an isotopic response, described as a type of Koebner phenomenon. Ionizing radiation itself can profoundly affect the skin. Both early and late changes can present, which typify the progression of changes following irradiation of the skin. Herein, we report a rare case of labetalol-associated lichen planus pemphigoides that occurred at the site treated with radiation for a soft tissue malignancy after 19 years as a result of an isotopic response. The rash was well-controlled after therapy and kept a 4-year remission. The same skin reaction recurred after the reintroduction of labetalol 4 years later.
Assuntos
Anti-Hipertensivos/efeitos adversos , Labetalol/efeitos adversos , Líquen Plano/induzido quimicamente , Penfigoide Bolhoso/induzido quimicamente , Lesões por Radiação , Idoso de 80 Anos ou mais , Toxidermias/patologia , Humanos , Líquen Plano/patologia , Masculino , Penfigoide Bolhoso/patologiaRESUMO
is missing (Short communication).
Assuntos
Líquen Plano/patologia , Penfigoide Bolhoso/patologia , Pele/patologia , Terminologia como Assunto , Glucocorticoides/uso terapêutico , Humanos , Imunossupressores/uso terapêutico , Líquen Plano/classificação , Líquen Plano/tratamento farmacológico , Masculino , Pessoa de Meia-Idade , Penfigoide Bolhoso/classificação , Penfigoide Bolhoso/tratamento farmacológico , Indução de Remissão , Pele/efeitos dos fármacos , Resultado do TratamentoRESUMO
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.
Assuntos
Líquen Plano , Mucosa , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Corticosteroides/uso terapêutico , Líquen Plano/tratamento farmacológico , Líquen Plano/patologia , Líquen Plano/imunologia , Líquen Plano/diagnóstico , Mucosa/patologia , Mucosa/imunologia , Penfigoide Bolhoso/imunologia , Penfigoide Bolhoso/tratamento farmacológico , Penfigoide Bolhoso/patologia , Penfigoide Bolhoso/diagnósticoRESUMO
It is well known that viral infections play a relevant role in inducing or protecting from autoimmune diseases, thus representing a major environmental factor in the disruption of the immune system in genetically susceptible individuals. Since the beginning of the Covid-19 pandemic a great number of clinical and epidemiological studies have demonstrated that SARS-CoV-2 infection is no exception to the rule by interfering on many different levels in the normal functioning of our immune system. Even though a growing number of case series and case reports has been cited in the literature linking the infection to the new onset of autoimmune diseases, to date very little has been reported concerning a possible correlation between the virus and the clinical resolution of any kind of autoimmune pathology. Here we describe an interesting case of abrupt and unexpected resolution of Lichen planus pemphigoides mucocutaneous lesions in a fully vaccinated patient after a mildly symptomatic SARS-CoV-2 respiratory infection and we speculate on the possible underlying mechanisms correlating the two events.
Assuntos
Doenças Autoimunes , COVID-19 , Líquen Plano , Humanos , Pandemias , SARS-CoV-2RESUMO
While the advent of immune-checkpoint inhibitors has revolutionized cancer therapy, immune-related adverse effects (irAEs) have also been on the rise. Cutaneous toxicities are among the most common irAEs, especially in the context of programmed cell death protein-1 (PD-1) inhibitors like pembrolizumab. Herein, we report a case of anti-PD-1-induced lichen planus pemphigoides (LPP)-a rare autoimmune blistering disorder with characteristics of both lichen planus and bullous pemphigoid. To our knowledge, this is the first reported case of LPP following anti-PD-1 therapy for metastatic adrenocortical cancer. Recognizing that LPP is within the spectrum of irAEs is important, especially as the indications for immunotherapy grow to include rarer malignancies like adrenocortical cancer. In addition to our case presentation, we also provide a comprehensive review of the literature surrounding immunotherapy-induced LPP-highlighting key characteristics towards the early recognition and clinical management of this cutaneous irAE.
RESUMO
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.
RESUMO
Pemphigoid diseases comprise a heterogeneous group of subepidermal autoimmune blistering dermatoses characterized by autoantibodies against structural proteins of the dermal-epidermal junction. Recent decades have witnessed a significant 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.
Assuntos
Epidermólise Bolhosa Adquirida , Líquen Plano , Penfigoide Bolhoso , Humanos , Penfigoide Bolhoso/diagnóstico , Estudos Prospectivos , AutoanticorposRESUMO
Lichen planus is a chronic papulosquamous eruption of the skin, scalp, nails, and mucous membranes. "Pruritic, purple, polygonal, planar, papules, plaques" are the traditional six "P's" of lichen planus. We describe an unusual case of lichen planus presenting as cellulitis. A 64-year-old lady with a past medical history of pyoderma gangrenosum, inclusion body myositis, and chronic kidney disease presented with a two-week history of swelling, erythema, tenderness, hyperkeratotic plaques, and blisters on the medial aspect of both thighs. She had a previous history of pyoderma gangrenosum exacerbations with similar presentations; however, current lesions were different from prior presentations. We considered the differential diagnoses of bacterial cellulitis versus pyoderma gangrenosum exacerbation. Due to the difference in these lesions from previous episodes, the patient was empirically treated for bacterial cellulitis with intravenous cefepime and linezolid. The infectious diseases team was consulted and valacyclovir was added to cover for possible herpes infection, with no improvement in symptomatology. Dermatology was then consulted, and a clinical diagnosis of psoriasiform dermatitis was made. A skin biopsy was obtained and the patient was started on prednisone. There was an immediate improvement in the papules within 24 hours. The papules cleared, leaving behind violaceous flat plaques, clinically diagnosed as lichen planus. The affected area was shrinking as compared to previous examinations. The skin biopsy was reported as chronic psoriasiform dermatitis with the main differential of lichen planus. The patient was discharged home on a tapering dose of oral prednisone, topical clobetasol, and oral moxifloxacin. This case demonstrates the importance of familiarity with rare clinical subtypes as a suspicion for lichen planus. The vesiculobullous subtype of lichen planus, as seen in this patient, tends to present as blisters and cellulitis from infection of the bullae. Treatment of the infection alone is not enough and steroids are essential. This knowledge helps change management, allows for earlier improvement and better patient outcomes.
RESUMO
Immune checkpoint inhibitor therapy nowadays became a treatment for a wide range of cancers, and may be responsible for various dermatologic adverse effects, including bullous eruptions. In our report, we present a case of late-onset immunotherapy-induced eruption in a 62-year-old woman treated with anti-programmed cell death-L1 agent durvalumab for metastatic squamous cell carcinoma. Diagnosed as lichenoid dermatitis upon initial presentation, this eruption evolved into necrotic bullous dermatitis after several weeks of phototherapy, with histology and direct immunofluorescence study favoring lichen planus pemphigoides. Thus, this case may be regarded as durvalumab-induced lichenoid dermatitis with phototherapy-triggered progression to necrotic lichen planus pemphigoides-like eruption. The patient's eruption responded to oral prednisone and immunotherapy interruption. Interestingly, durvalumab reintroduction in this patient led to recurrent lichenoid dermatitis without bullous component. This case of immunotherapy skin toxicity is rather distinctive by its clinical and histopathologic features, with phototherapy as an additional triggering factor.
RESUMO
Decorative tattooing is very popular worldwide and is associated with cutaneous complications, ranging from infections to localized or generalized skin reactions. We report a case of a patient presenting with generalized violaceous pruritic papular lesions 1 month after obtaining a black ink tattoo. Histological examination of a papular lesion distal to the tattoo site showed focal band-like lymphocytic infiltrate. He subsequently developed bullae over the papular lesions, with elevated serum BP180 antibody levels. A diagnosis of generalized lichen planus and lichen planus pemphigoides was made. He responded to treatment with potent topical corticosteroids and ciclosporin. We also reviewed the presentation and treatment of published cases of lichenoid reactions in the literature. With the increasing popularity of tattoos, awareness of this potential complication and possible treatments is important.
Assuntos
Líquen Plano , Tatuagem , Administração Cutânea , Humanos , Líquen Plano/tratamento farmacológico , Líquen Plano/etiologia , Masculino , Pele , Tatuagem/efeitos adversosRESUMO
Lichen planus pemphigoides (LPP) is a very rare autoimmune sub-epidermal blistering disease associated with lichenoid skin changes. Initially thought to be a mere variant of more common inflammatory dermatoses, particularly Bullous Pemphigoid (BP) or Lichen Planus (LP), a growing body of evidence suggests that it is a disease entity in its own right. In common with a range of autoimmune blistering diseases, including BP, pemphigoid gestationis (PG), mucous membrane pemphigoid (MMP) and linear IgA dermatosis (LAD), a key feature of the disease is the development of autoantibodies against type XVII collagen (COL17). However, accurately establishing the diagnosis is dependent on a careful correlation between the clinical, histological and immunological features of the disease. Therefore, we present an up to date summary of the epidemiology and etiopathogenesis of LPP, before illustrating the predisposing and precipitating factors implicated in the development of the disease. In addition to a selective literature search, we compare reports of potential drug-induced cases of LPP with pharmacovigilance data available via OpenVigil. We subsequently outline the cardinal clinical features, important differential diagnoses and current treatment options. We conclude by demonstrating that an improved understanding of LPP may not only lead to the development of novel treatment strategies for the disease itself, but may also shed new light on the pathophysiology of more common and treatment-refractory autoimmune blistering diseases.