814 results match your criteria Pemphigus Paraneoplastic


Current and Innovated Managements for Autoimmune Bullous Skin Disorders: An Overview.

J Clin Med 2022 Jun 19;11(12). Epub 2022 Jun 19.

Department of Dermatology, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung 807378, Taiwan.

Autoimmune bullous skin disorders are a group of disorders characterized by the formation of numerous blisters and erosions on the skin and/or the mucosal membrane, arising from autoantibodies against the intercellular adhesion molecules and the structural proteins. They can be classified into intraepithelial or subepithelial autoimmune bullous dermatoses based on the location of the targeted antigens. These dermatoses are extremely debilitating and fatal in certain cases, depending on the degree of cutaneous and mucosal involvement. Read More

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Ruxolitinib, a JAK1/2 Inhibitor as Treatment for Paraneoplastic Pemphigus: A Case Report.

Acta Derm Venereol 2022 06 9;102:adv00732. Epub 2022 Jun 9.

Department of Dermatology, Peking University First Hospital, Beijing 100034, China.

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Indolent T-lymphoblastic proliferation: A systematic review of the literature analyzing the epidemiologic, clinical, and pathologic features of 45 cases.

Int J Lab Hematol 2022 May 16. Epub 2022 May 16.

Department of Pathology, University of California San Francisco, San Francisco, California, USA.

An indolent T-lymphoblastic proliferation (iT-LBP) is a rare benign disorder characterized by an abnormal expansion of immature T-cells, which morphologically can mimic malignancy. Since the first case was described in 1999, dozens more have been reported in the literature. However, the epidemiologic, clinical, pathologic, and biologic features of this disease have not been well described. Read More

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Paraneoplastic myasthenia gravis and pemphigus associated with follicular dendritic cell sarcoma leading to cardiorespiratory collapse in a 7-year-old.

Pediatr Blood Cancer 2022 Apr 29:e29723. Epub 2022 Apr 29.

Division of Rheumatology, Department of Pediatrics, Perelman School of Medicine, Children's Hospital of Philadelphia, University of Pennsylvania, Philadelphia, Pennsylvania, USA.

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Antibody-Negative Paraneoplastic Autoimmune Multiorgan Syndrome (PAMS) in a Patient with Follicular Lymphoma Accompanied by an Excess of Peripheral Blood CD8+ Lymphocytes.

Curr Oncol 2022 03 28;29(4):2395-2405. Epub 2022 Mar 28.

Skin Cancer Center, Department of Dermatology, Ruhr-University Bochum, 44791 Bochum, Germany.

Paraneoplastic autoimmune multiorgan syndrome (PAMS) is a life-threatening autoimmune disease associated with malignancies. Here, we present a patient initially misdiagnosed with "chronic" Stevens-Johnson syndrome. Over a year later, the patient was diagnosed with stage IV follicular lymphoma and treated with an anti-CD20 antibody. Read More

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Thoracoabdominal computed tomography neoplasia detection in patients with paraneoplastic pemphigus: the importance of collaboration between specialists.

Acta Dermatovenerol Alp Pannonica Adriat 2022 Mar;31(1):1-5

Department of Clinical and Molecular Sciences, Dermatology Clinic, Polytechnic Marche University, Ancona, Italy.

Introduction: Paraneoplastic pemphigus (PNP) is an autoimmune disorder that is almost always linked to an underlying neoplasia. General radiologists are usually not aware of what kind of neoplasia can be associated with PNP. Therefore, this study evaluates the effect of a dermatology lecture on radiologists' neoplasia diagnosis performance. Read More

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Atypical Paraneoplastic Pemphigus Without Mucosal Involvement.

Actas Dermosifiliogr 2022 05 19;113(5):519-521. Epub 2021 Nov 19.

Dermatology Department, Centro Hospitalar e Universitário do Porto, Porto, Portugal; Instituto de Ciências Biomédicas Abel Salazar, University of Porto, Porto, Portugal.

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The Immunogenetics of Autoimmune Blistering Diseases.

Adv Exp Med Biol 2022 ;1367:173-212

Division of Dermatology, Rush University Medical Center, Chicago, USA.

Dermatological conditions constituting the group of autoimmune blistering diseases (AIBD) are characterized by loss of immunotolerance and humoral, as well as cellular, autoimmune responses that result in the development of bullae and erosions on the skin and mucous membranes. AIBDs are broadly categorized into pemphigus and pemphigoid classes with several distinct subtypes amongst them. Advances in genetics have allowed for the study and identification of alleles, and even single nucleotide polymorphisms, that harbor increased susceptibility or confer protection for the development of these conditions. Read More

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Clinical characteristics and prognosis of patients with Castleman disease in a Chinese hospital: paraneoplastic pemphigus is an independent risk factor.

Am J Transl Res 2022 15;14(2):1051-1059. Epub 2022 Feb 15.

Department of Urology, The First Affiliated Hospital of Nanjing Medical University Nanjing 210029, Jiangsu Province, China.

Objective: Castleman disease (CD) is a rare lymphoproliferative disorder with limited clinical research data. This study aimed to investigate the clinical manifestations, pathologic features, and prognostic factors of CD.

Methods: The clinicopathological data of 54 patients with CD hospitalized in the First Affiliated Hospital of Nanjing Medical University were retrospectively analyzed. Read More

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February 2022

Bronchiolitis Obliterans With Anti-Epiplakin Antibodies in a Boy With Paraneoplastic Pemphigus.

Pediatrics 2022 03;149(3)

Dermatology Department.

Paraneoplastic pemphigus is a rare and severe autoimmune blistering disease characterized by a recalcitrant and severe mucositis, and polymorphic cutaneous lesions, associated with benign and malignant neoplasms. Paraneoplastic pemphigus is caused by production of autoantibodies against various epidermal proteins involved in cell adhesion. Bronchiolitis obliterans (BO) is one of the leading causes of mortality in these patients. Read More

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Utility of IgG4 immunohistochemistry detection in pemphigus diagnosis.

SAGE Open Med Case Rep 2022 19;10:2050313X211072982. Epub 2022 Jan 19.

Consulta de Dermatología, Luminox Skin Centre, Torre Noox, Ciudad de México, México.

Pemphigus includes a group of blistering autoimmune diseases that affect the skin and mucosa, characterized by the formation of epidermal bullous and the presence of antibodies against binding proteins. Pemphigus is classified according to clinical presentation, target molecule, and IgG production as pemphigus vulgaris, foliaceous, IgA-pemphigus, and paraneoplastic pemphigus. Thus, the identification of autoantibodies class and site of deposition is mandatory. Read More

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January 2022

Clinical and pathological features of bronchiolitis obliterans requiring lung transplantation in paraneoplastic pemphigus associated with Castleman disease.

Clin Respir J 2022 Mar 21;16(3):173-181. Epub 2022 Jan 21.

National Center for Respiratory Medicine, Beijing, China.

At A Glance: Bronchiolitis obliterans in paraneoplastic pemphigus associated with Castleman disease possesses the progressive nature even when it is treated with intensive medical therapy. Antibodies were at least in low titers before the Lung transplant and remain negative after the procedure. Explanted lungs showed coexistence of cellular destructive bronchiolitis and constrictive bronchiolitis. Read More

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Reflectance confocal microscopic presentation of paraneoplastic pemphigus.

Australas J Dermatol 2022 Feb 21;63(1):142-144. Epub 2021 Dec 21.

Department of Dermatology, Wuhan No. 1 Hospital, Wuhan, China.

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February 2022

Eosinophilic dermatosis in a patient with chronic lymphocytic leukemia: a rare case report.

Acta Dermatovenerol Alp Pannonica Adriat 2021 Dec;30(4):161-164

Hematological Laboratory, Second Department of Internal Medicine, Aristotle University, Hippokration Hospital, Thessaloniki, Greece.

Chronic lymphocytic leukemia (CLL) is a type of malignant lymphoproliferative disorder characterized by a rapid and uncontrolled increase in lymphoid cells, mostly monoclonal B-cells (B-CLL). Patients with CLL may present cutaneous lesions that can be classified as either "specific" or "non-specific." In CLL patients, specific skin eruptions arise from leukemic cell infiltration, recognized histopathologically in tissue sample biopsy. Read More

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December 2021

A gene-centric approach to biomarker discovery identifies transglutaminase 1 as an epidermal autoantigen.

Proc Natl Acad Sci U S A 2021 12;118(51)

Center for Molecular Medicine, Department of Medicine (Solna), Karolinska Institutet, Stockholm 171 76, Sweden.

Autoantigen discovery is a critical challenge for the understanding and diagnosis of autoimmune diseases. While autoantibody markers in current clinical use have been identified through studies focused on individual disorders, we postulated that a reverse approach starting with a putative autoantigen to explore multiple disorders might hold promise. We here targeted the epidermal protein transglutaminase 1 (TGM1) as a member of a protein family prone to autoimmune attack. Read More

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December 2021

Epidemiology of Pemphigus.

JID Innov 2021 Mar 20;1(1):100004. Epub 2021 Feb 20.

Lűbeck Institute of Experimental Dermatology, University of Lübeck, Lübeck, Germany.

Pemphigus is an epidemiologically heterogeneous group of autoimmune bullous diseases comprising pemphigus vulgaris (PV), pemphigus foliaceus, paraneoplastic pemphigus, IgA pemphigus, and pemphigus herpetiformis. Recently, our knowledge about the frequency of pemphigus, which is highly variable between different populations, has considerably expanded, and the first non-HLA genes associated with PV have been identified. In addition, a variety of comorbidities, including other autoimmune diseases, hematological malignancies, and psoriasis, have been described in this variant. Read More

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Successful treatment of paraneoplastic pemphigus and bronchiolitis obliterans associated with follicular lymphoma with obinutuzumab and bendamustine.

Curr Probl Cancer 2022 04 19;46(2):100813. Epub 2021 Nov 19.

Department of Hematology, Japanese Red Cross Kyoto Daiichi Hospital, Kyoto, Japan.

We herein report the rare case of a 72-year-old female who presented with paraneoplastic pemphigus (PNP) and bronchiolitis obliterans (BO) associated with follicular lymphoma (FL), who was successfully treated with obinutuzumab (GA101; G) and bendamustine (B). The patient had severe erosive stomatitis and bilateral conjunctival hyperemia that persisted for more than 6 months. A huge mass was found in the abdominal cavity, and a biopsy revealed grade 1 FL (stage IV). Read More

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Invasive breast cancer found in a patient with new-onset pemphigus foliaceus.

JAAD Case Rep 2021 Dec 23;18:67-70. Epub 2021 Oct 23.

University of Chicago, Pritzker School of Medicine, Chicago, Illinois.

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December 2021

Comorbid diseases associated with pemphigus: a case-control study.

J Dtsch Dermatol Ges 2021 11 3;19(11):1613-1619. Epub 2021 Nov 3.

Department of Experimental, Diagnostic and Specialty Medicine - Dermatology - IRCCS Policlinico di Sant'Orsola, Bologna, Italy.

Background And Objectives: Pemphigus has been associated with physical and psychiatric comorbid diseases. This study aims to further investigate these associations in patients with pemphigus, and to analyze the relationships of comorbid conditions with sex and age, pemphigus disease area index score, diagnostic delay and cutaneous/mucous involvement.

Patients And Methods: Patients with pemphigus were matched by age, gender and area of residence with eight controls each. Read More

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November 2021

A Rare Lymphoproliferative Disease: Castleman Disease

Turk J Haematol 2021 12 1;38(4):314-320. Epub 2021 Nov 1.

Başkent University Faculty of Medicine, Department of Hematology, Ankara, Turkey

Castleman disease is a rare lymphoproliferative disease also known as angiofollicular lymph node hyperplasia. It is classified as hyaline vascular and plasmacytic variants histologically but characteristics of both types can coexist. Most unicentric cases of the disease are hyaline vascular while most multicentric cases are of the plasmacytic type. Read More

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December 2021

Plectin in Skin Fragility Disorders.

Cells 2021 10 14;10(10). Epub 2021 Oct 14.

Department of Dermatology, Faculty of Medicine, Medical Center-University of Freiburg, 79104 Freiburg, Germany.

Plectin is a multi-faceted, 500 kDa-large protein, which due to its expression in different isoforms and distinct organs acts diversely as a cytoskeletal crosslinker and signaling scaffold. It functions as a mediator of keratinocyte mechanical stability in the skin, primarily through linking intermediate filaments to hemidesmosomes. Skin fragility may occur through the presence of mutations in the gene encoding for plectin, , or through the presence of autoantibodies against the molecule. Read More

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October 2021

An Updated Review of Pemphigus Diseases.

Medicina (Kaunas) 2021 Oct 9;57(10). Epub 2021 Oct 9.

Department of Dermatology, College of Medicine, University of Florida, Gainesville, FL 32606, USA.

Clinicians may encounter a variety of skin conditions that present with vesiculobullous lesions in their everyday practice. Pemphigus vulgaris, pemphigus foliaceus, IgA pemphigus, and paraneoplastic pemphigus represent the spectrum of autoimmune bullous dermatoses of the pemphigus family. The pemphigus family of diseases is characterized by significant morbidity and mortality. Read More

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October 2021

Comprehensive review on the pathophysiology, clinical variants and management of pemphigus (Review).

Exp Ther Med 2021 Nov 20;22(5):1335. Epub 2021 Sep 20.

Department of Oral Medicine and Oral Dermatology, 'Grigore T. Popa' University of Medicine and Pharmacy, 700115 Iași, Romania.

Pemphigus represents a group of chronic inflammatory disorders characterized by autoantibodies that target components of desmosomes, leading to the loss of intercellular adhesion between keratinocytes and causing intraepithelial blistering. The pemphigus group consists of four main clinical types with several variants: pemphigus vulgaris (with pemphigus vegetans and pemphigus herpetiformis as variants), pemphigus foliaceus, paraneoplastic pemphigus and IgA pemphigus (with two clinical variants: intraepidermal neutrophilic IgA dermatosis and subcorneal pustular dermatosis). Genetic factors are involved in the pathogenesis, with HLA-DR4 (DRB1*0402) and HLA-DRw6 (DQB1*0503) allele more common in patients with pemphigus vulgaris, HLA class II DRB1*0344 and HLA Cw*1445 correlated with paraneoplastic pemphigus, and HLA-DRB1*04:01, HLA-DRB1*04:06, HLA-DRB1*01:01, HLA-DRB1*14, associated with a higher risk of developing pemphigus foliaceus. Read More

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November 2021

Bilateral lung transplantation for Castleman disease with end-stage bronchiolitis obliterans.

Clin Transplant 2022 01 21;36(1):e14496. Epub 2021 Oct 21.

Wuxi Lung Transplant Center, Department of Thoracic Surgery, Wuxi People's Hospital affiliated to Nanjing Medical University, Wuxi, China.

Bronchiolitis obliterans (BO) is a severe complication of Castleman disease (CD), a rare lymphoproliferative disease with unclear pathogenesis. Currently, there are no reports on the safety or outcomes of bilateral lung transplantation in patients with BO due to CD. This study aimed to characterize the clinical manifestations and features of BO and CD. Read More

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January 2022

Therapy of paraneoplastic pemphigus with Rituximab: A case report.

Dermatol Ther 2021 11 6;34(6):e15144. Epub 2021 Oct 6.

Department of Dermatology, Wuhan No. 1 Hospital, Wuhan, China.

We report a case of 39-year-old female patient with paraneoplastic pemphigus (PNP) associated with thymoma treated with rituximab plus corticosteroids and methotrexate. After rituximab therapy for 8 weeks, oral ulcerations had cured, lesions on the trunk and limbs improved. Rituximab may be useful for PNP therapy, but further studies are necessary to confirm this hypothesis. Read More

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November 2021

Paraneoplastic pemphigus associated with interdigitating dendritic cell sarcoma.

Indian J Dermatol Venereol Leprol 2021 Jan-Feb;88(1):80-82

Department of Dermatology, The Second Affiliated Hospital of Zhejiang University, Hangzhou, China.

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Anti-laminin 332 antibody detection using biochip immunofluorescence microscopy in a real-life cohort of Italian patients with mucous membrane pemphigoid.

Eur J Dermatol 2021 Aug 26. Epub 2021 Aug 26.

Section of Dermatology, Department of health Sciences (DISSAL), University of Genoa, Genoa, Italy.

Mucous membrane pemphigoid (MMP) with anti-laminin 332 autoantibodies may be associated with malignancies, however, current serological assays have considerable limitations. At present, no commercial test for anti-laminin 332 antibodies is available, restricting the diagnosis to specialized laboratories worldwide. Biochip immunofluorescence microscopy has shown promising results in selected cohorts of laminin 332-MMP patients. Read More

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