771 results match your criteria Pemphigus Paraneoplastic

A Case of Paraneoplastic Pemphigus as a Preceding Manifestation of Underlying Follicular Lymphoma Treated with R-CHOP.

Ann Dermatol 2021 Jun 4;33(3):271-274. Epub 2021 May 4.

Department of Dermatology, Seoul National University Bundang Hospital, Seongnam, Korea.

Paraneoplastic pemphigus is a rare, life-threatening disorder associated with an underlying neoplasm, which presents with painful stomatitis and polymorphous skin lesions. Successful diagnosis of paraneoplastic pemphigus can lead to the diagnosis and treatment of the underlying malignancy. However, involvement of the respiratory system is typically unresponsive to treatment. Read More

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Pemphigus Vulgaris After COVID-19: a Case of Induced Autoimmunity.

SN Compr Clin Med 2021 May 27:1-5. Epub 2021 May 27.

Department of Pathology, Federal University of Pernambuco, Recife, PE Brazil.

Bullae autoimmune diseases can be induced by environmental factors in a genetically susceptible individual, and viruses may be important triggers for this process. Coronavirus disease 2019 (COVID-19) is a multisystemic disease known for developing many types of skin lesions. However, little is known about post-COVID-19 manifestations. Read More

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A Case of Severe Refractory Pemphigus Vulgaris in a Patient With Stable Esophageal Malignancy.

Cureus 2021 Apr 20;13(4):e14576. Epub 2021 Apr 20.

Rheumatology, Rutgers University, Newark, USA.

Pemphigus is a broad term that is used to describe a group of bullous autoimmune diseases affecting the skin and mucous membranes; the pathogenesis involves autoantibodies directed against various cell junction desmosomal proteins. In patients with a history of malignancy who present with bullous lesions, the differential diagnosis may include, but is not limited to, paraneoplastic pemphigus (PNP) and pemphigus vulgaris (PV) secondary to a primary autoimmune process, or induced by chemotherapy or radiation therapy. In this report, we present a case of refractory PV in a patient with stable esophageal cancer, five years after undergoing radiation therapy. Read More

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Paraneoplastic pemphigus associated with post-transplant lymphoproliferative disorder after small bowel transplantation.

Pediatr Transplant 2021 May 20:e14023. Epub 2021 May 20.

Department of Pediatric Gastroenterology, University Medical Center Groningen, Groningen, The Netherlands.

Background: PNP is a malignancy-associated autoimmune mucocutaneous syndrome due to autoantibodies against plakins, desmogleins, and other components of the epidermis and basement membrane of epithelial tissues. PNP-causing malignancies comprise mainly lymphoproliferative and hematologic neoplasms. PNP is extremely rare, especially in children. Read More

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Paraneoplastic pemphigus caused by pre-existing stroma-rich variant of Castleman disease: from a pathologist's point of view.

BMJ Case Rep 2021 May 11;14(5). Epub 2021 May 11.

Department of Cellular Pathology, North Bristol NHS Trust, Westbury on Trym, UK.

A young woman presented with mucocutaneous blisters and ulcerating lesions, and was diagnosed with erythrodermic pemphigus complicated by bronchiolitis obliterans. Her clinical condition did not improve on immunosuppressive therapy. She had a history of an asymptomatic retroperitoneal mass, presumed to be a dermoid cyst, followed up clinically. Read More

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Paraneoplastic pemphigus without mucosal lesions in a patient with ovarian cancer.

Int J Dermatol 2021 May 11. Epub 2021 May 11.

Center for Dermatology, Department of Pathology and Laboratory Medicine, Rutgers Robert Wood Johnson Medical School, Somerset, NJ, USA.

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Toxic epidermal necrolysis-like presentation of paraneoplastic pemphigus due to underlying thymoma: A clinical conundrum.

J Postgrad Med 2021 Apr-Jun;67(2):119-121

Department of Dermatology, Government Medical College and Hospital, Chandigarh, India.

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Paraneoplastic pemphigus: A concern for anaesthetists in resource constrained settings.

Eur J Anaesthesiol 2021 May;38(5):562-565

From the Department of Cardiothoracic and Vascular Anaesthesiology, Manmohan Cardiothoracic Vascular and Transplant Center (SS, PB, BP), Department of Anaesthesiology, Tribhuvan University Teaching Hospital, Maharajgunj Medical Campus, Tribhuvan University Institute of Medicine, Kathmandu, Nepal (SD) and Department of Breast Surgery, Jyoban Hospital of Tokiwa Foundation, Iwaki, Fukushima, Japan (AO).

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Castleman disease and paraneoplastic pemphigus in a pregnant woman: A case report.

Beibei Cui Hui Lin

Medicine (Baltimore) 2021 Apr;100(13):e24990

Department of Rheumatology and Immunology, West China hospital, Sichuan University, Chengdu, Sichuan Province, PR China.

Rationale: Orogenital ulcers can be observed in various conditions, such as Behcet disease, infection and also paraneoplastic pemphigus (PNP). Castleman disease (CD), which is a rare cause of paraneoplastic pemphigus represents a heterogenous lymphoproliferative disorder of unknown etiology. Paraneoplastic pemphigus associated with CD in pregnancy is rare and has not been reported yet. Read More

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Autoantibodies to desmocollin 3 in pemphigus exclusively recognize calcium-dependent epitope in extracellular domain 2.

J Invest Dermatol 2021 Mar 22. Epub 2021 Mar 22.

Department of Dermatology, Kurume University School of Medicine, Japan.

Pemphigus is a group of autoimmune bullous diseases characterized by the presence of autoantibodies against adhesion molecules, desmogleins (Dsg) and desmocollins (Dsc). Pathogenicity of anti-Dsc3 antibodies in pemphigus has been demonstrated, however its characteristics have not yet been elucidated. We aimed to analyze the characteristics of anti-Dsc3 antibodies using Dsc3 domain-swapped Dsg2 molecules in which the prosequence and five extracellular (EC) domains of Dsg2 were replaced with the corresponding domains of human Dsc3. Read More

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Successful use of intravenous and intralesional rituximab in paraneoplastic pemphigus with Castleman's disease.

Int J Dermatol 2021 Mar 15. Epub 2021 Mar 15.

Department of Dermatology, Rajiv Gandhi Medical College and Chhatrapati Shivaji Maharaj Hospital, Thane, Maharashtra, India.

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Paraneoplastic pemphigus: Revised diagnostic criteria based on literature analysis.

J Cutan Pathol 2021 Mar 14. Epub 2021 Mar 14.

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

Background: Paraneoplastic pemphigus (PNP) is a rare autoimmune bullous disease classically associated with an underlying neoplasm. The heterogeneous clinical and histopathologic features of the disease make diagnosis challenging for clinicians. There are no formally accepted diagnostic criteria, and newer techniques for identifying antibodies directed against plakin proteins have largely replaced immunoprecipitation, the historic gold standard. Read More

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Intraepithelial autoimmune blistering dermatoses: Clinical features and diagnosis.

J Am Acad Dermatol 2021 Jun 5;84(6):1507-1519. Epub 2021 Mar 5.

Department of Dermatology, Mayo Clinic, Rochester, Minnesota; Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota.

Intraepithelial autoimmune blistering dermatoses are a rare group of skin disorders characterized by the intraepithelial disruption of intercellular connections through the action of autoantibodies. The first article in this continuing medical education series explores the background, epidemiology, clinical features, and diagnostic criteria of each of the major intraepithelial autoimmune blistering dermatoses, including pemphigus foliaceus, pemphigus erythematosus, pemphigus herpetiformis, fogo selvagem, pemphigus vulgaris, pemphigus vegetans, drug-induced pemphigus, IgA pemphigus, IgG/IgA pemphigus, and paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome. Read More

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Intraepithelial autoimmune bullous dermatoses disease activity assessment and therapy.

J Am Acad Dermatol 2021 Jun 5;84(6):1523-1537. Epub 2021 Mar 5.

Epiphany Dermatology, Dallas, Texas. Electronic address:

Intraepithelial autoimmune blistering dermatoses are a rare group of skin disorders characterized by disruptions of inter-keratinocyte connections within the epidermis through the action of autoantibodies. The second article in this continuing medical education series presents validated disease activity scoring systems, serologic parameters of disease, treatments, and clinical trials for pemphigus and its subtypes. Read More

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Clinical characteristics and immunological abnormalities of Castleman disease complicated with autoimmune diseases.

J Cancer Res Clin Oncol 2021 Jul 5;147(7):2107-2115. Epub 2021 Feb 5.

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

Purpose: To explore the clinical features and immunological mechanisms of Castleman disease (CD) complicated with autoimmune diseases (AID).

Methods: We explored the prevalence and clinical manifestations of CD complicated with AID by reviewing clinical, pathological, and laboratory data of 40 CD patients retrospectively, and then explored abnormal immune mechanisms in the co-existence of the two entities by monitoring lymphocyte subsets in peripheral blood.

Results: Paraneoplastic pemphigus, autoimmune hemolytic anemia, Sjogren's syndrome, myasthenia gravis, and psoriasis were found to be coexisted with CD in 9/40 (22. Read More

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Cutaneous involvement as initial presentation of multicentric plasmacytic Castleman disease.

J Cutan Pathol 2021 May 7;48(5):701-705. Epub 2021 Mar 7.

Department of Pathology, St. Vincent Evansville Medical Center, Evansville, Indiana, USA.

Castleman disease (CD) is an unusual lymphoproliferative disorder characterized by multiple lymphadenopathy accompanied by marked systemic inflammatory symptoms. CD can be unicentric (UCD) or multicentric (MCD), and it can be classified into three types based on histopathology: hyaline vascular type, plasma cell type, and mixed hyaline vascular and plasma cell type. CD involving skin is an unusual clinical manifestation. Read More

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Paraneoplastic pemphigus associated with small lymphocytic lymphoma: A case report.

Medicine (Baltimore) 2021 Jan;100(2):e24039

The second Clinical Medical College of Zhejiang Chinese Medical University, Hangzhou, China.

Introduction: Paraneoplastic pemphigus (PNP) is a life-threatening autoimmune blistering disease associated with underlying neoplasms. Currently, this disease is very difficult to treat.

Patient Concerns: We reported a rare case of paraneoplastic pemphigus associated with small lymphocytic lymphoma responsive to desmoglein 3 (Dsg3) and bullous pemphigoid (BP) antigen 180. Read More

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January 2021

Radiotherapy-Associated Paraneoplastic Pemphigus and Its Complications in a Patient with Nasopharyngeal Carcinoma.

Eur J Case Rep Intern Med 2020 12;7(12):002026. Epub 2020 Nov 12.

Department of Pulmonology & Critical Care, Shifa International Hospital, Islamabad, Pakistan.

Paraneoplastic pemphigus arising in association with non-haematological cancers is extremely rare, and there are no reported cases of a patient developing this in the setting of nasopharyngeal carcinoma and only 2 reported cases of patients developing this in response to radiotherapy. Here, we present the case of a patient who developed radiotherapy-associated paraneoplastic pemphigus in the setting of nasopharyngeal carcinoma and who then developed multiple complications.

Learning Points: Paraneoplastic pemphigus has a remarkably similar clinical picture to other dermatologic diseases (for example, pemphigus vulgaris), and therefore, it is easy to miss the diagnosis. Read More

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November 2020

Drug-induced pemphigus: A systematic review of 170 patients.

Int Immunopharmacol 2021 Mar 5;92:107299. Epub 2021 Jan 5.

Autoimmune Bullous Diseases Research Center, Tehran University of Medical Sciences, Tehran, 009821 Iran.

Pemphigus encompasses a rare heterogeneous group of autoimmune blistering diseases characterized by cutaneous and/or mucosal blistering. Multiple factors, such as some specific types of drugs, have been found to be involved in the induction of pemphigus. Here, we have designed a systematic review by searching PubMed/Medline and Embase databases to find the drugs, involved in pemphigus induction and exacerbation (updated on 19 August 2019). Read More

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Multidisciplinary diagnosis and treatment of recurrent follicular dendritic cell sarcoma in abdomen: A case report.

Medicine (Baltimore) 2020 Dec;99(51):e23588

Department of General Surgery.

Rationale: Follicular dendritic cell sarcoma (FDCS) is a rare malignant tumor derived from follicular dendritic cells, and is often associated with Castleman disease. Here we present a rare case of paraneoplastic pemphigus (PNP) with FDCS which required multidisciplinary approach for the diagnosis and treatment.

Patient Concerns: A 28-year-old Chinese female had FDCS recurrence, and primary clinical manifestation was PNP. Read More

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December 2020

Systemic steroids and risk of fecal-oral shedding and increased transmission of SARS-CoV-2 in pemphigus cases.

Clin Dermatol 2020 Nov - Dec;38(6):750-756. Epub 2020 Jun 19.

Department of Dermatology, JK Medical College & LN Hospital, Bhopal, India. Electronic address:

Pemphigus and its variants, viz., vulgaris, foliaceous, vegetans, Ig A pemphigus, paraneoplastic pemphigus and Senear-Usher syndrome are rare autoimmune blistering diseases of the skin and/or mucous membranes. The autoantibodies involved in the pathogenesis of pemphigus against desmoglein result in the breach of the skin and mucosal barrier, which acts as the first line of defence against pathogens. Read More

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December 2020

Localized pemphigus exacerbation associated with underlying breast cancer.

JAAD Case Rep 2020 Dec 17;6(12):1268-1270. Epub 2020 Oct 17.

Department of Health Sciences, Section of Dermatology, University of Florence, Florence, Italy.

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December 2020

New Clinicopathologic Scenarios of EBV+ Inflammatory Follicular Dendritic Cell Sarcoma: Report of 9 Extrahepatosplenic Cases.

Am J Surg Pathol 2021 Jun;45(6):765-772

Department of Pathology, Fudan University Shanghai Cancer Center.

EBV+ inflammatory follicular dendritic cell (FDC) sarcoma is an indolent malignant neoplasm of spindled FDCs with a rich lymphoplasmacytic infiltrate and a consistent association with Epstein-Barr virus (EBV). It occurs exclusively in the liver and spleen, with the exception of a few colonic examples. In this study, we report 9 extrahepatosplenic cases, including 4 occurring in previously undescribed sites, but all apparently anatomically related to the aerodigestive tract. Read More

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Deep pemphigus (pemphigus vulgaris, pemphigus vegetans and paraneoplastic pemphigus) in dogs, cats and horses: a comprehensive review.

BMC Vet Res 2020 Nov 23;16(1):457. Epub 2020 Nov 23.

Department of Clinical Sciences, College of Veterinary Medicine, North Carolina State University, Raleigh, NC, USA.

Pemphigus is the term used to describe a group of rare mucocutaneous autoimmune bullous diseases characterized by flaccid blisters and erosions of the mucous membranes and/or skin. When the autoantibodies target desmosomes in the deep layers of the epidermis, deep pemphigus variants such as pemphigus vulgaris, pemphigus vegetans and paraneoplastic pemphigus develop. In this article, we will review the signalment, clinical signs, histopathology and treatment outcome of pemphigus vulgaris, pemphigus vegetans and paraneoplastic pemphigus in dogs, cats and horses; where pertinent, we compare the animal diseases to their human homologue. Read More

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November 2020