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Paraneoplastic Pemphigus

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41. Prevalence of myasthenia gravis and associated autoantibodies in paraneoplastic pemphigus and their correlations with symptoms and prognosis. (PubMed)

Prevalence of myasthenia gravis and associated autoantibodies in paraneoplastic pemphigus and their correlations with symptoms and prognosis. Paraneoplastic pemphigus (PNP) involves multiple organs, but little is known about its neurological involvement.To investigate the symptoms, prognosis and profiles of associated autoantibodies in myasthenia gravis (MG), and their correlations in patients with PNP.Fifty-eight patients with PNP were assessed for myasthenic symptoms and laboratory evidence (...) . Serum autoantibodies against acetylcholine receptor (AChR), acetylcholinesterase (AChE), titin, ryanodine receptor (RyR) and muscle-specific kinase (MuSK) were measured by enzyme-linked immunosorbent assay. Patients with pemphigus vulgaris (PV), pemphigus foliaceus (PF), connective tissue disease (CTD) and non-PNP MG (NP-MG), and healthy donors, served as controls. These autoantibodies in PNP were also compared in the presence or absence of dyspnoea or muscle weakness. Cox regression and log-rank

2014 British Journal of Dermatology

42. Paraneoplastic pemphigus associated with inflammatory myofibroblastic tumour of the mediastinum: A favourable response to treatment and review of the literature. (PubMed)

Paraneoplastic pemphigus associated with inflammatory myofibroblastic tumour of the mediastinum: A favourable response to treatment and review of the literature. Paraneoplastic pemphigus (PNP) is an autoimmune blistering disorder that occurs in association with an underlying neoplasm. Inflammatory myofibroblastic tumour (IMT) is a rare low-grade sarcoma of fibroblasts and myofibroblasts associated with inflammatory cells, most commonly occurring in the lung. In this study, a case of PNP

2014 Australasian Journal of Dermatology

43. IgA pemphigus with paraneoplastic pemphigus-like clinical features showing IgA antibodies to desmoglein 1/3 and desmocollin 3, and IgG and IgA antibodies to the basement membrane zone. (PubMed)

IgA pemphigus with paraneoplastic pemphigus-like clinical features showing IgA antibodies to desmoglein 1/3 and desmocollin 3, and IgG and IgA antibodies to the basement membrane zone. A 79-year-old Japanese woman presented with severe recalcitrant erosions on her oral mucosa, resembling paraneoplastic pemphigus. Using indirect immunofluorescence, we detected IgA antibodies against the cell surface, and both IgG and IgA antibodies against the basement membrane zone. Immunoblotting showed

2013 Clinical & Experimental Dermatology

44. Pemphigus, Paraneoplastic (Overview)

Pemphigus, Paraneoplastic (Overview) Paraneoplastic Pemphigus: Background, Pathophysiology, Etiology Edition: No Results No Results Please confirm that you would like to log out of Medscape. If you log out, you will be required to enter your username and password the next time you visit. https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile&urlCache=aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvMTA2NDQ1Mi1vdmVydmlldw== processing > Paraneoplastic Pemphigus (...) Updated: Feb 07, 2019 Author: Lynne J Goldberg, MD; Chief Editor: Dirk M Elston, MD Share Email Print Feedback Close Sections Sections Paraneoplastic Pemphigus Overview Background Anhalt et al [ ] first described paraneoplastic pemphigus in 1990. The authors reported five patients with underlying neoplasms who developed oral erosions and bullous skin eruptions. Skin biopsy samples showed both suprabasal acantholysis and interface dermatitis. Direct immunofluorescence (DIF) testing and indirect

2014 eMedicine.com

45. Pemphigus, Paraneoplastic (Treatment)

Pemphigus, Paraneoplastic (Treatment) Paraneoplastic Pemphigus Treatment & Management: Medical Care, Surgical Care, Consultations Edition: No Results No Results Please confirm that you would like to log out of Medscape. If you log out, you will be required to enter your username and password the next time you visit. https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile&urlCache=aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvMTA2NDQ1Mi10cmVhdG1lbnQ= processing (...) > Paraneoplastic Pemphigus Treatment & Management Updated: Feb 07, 2019 Author: Lynne J Goldberg, MD; Chief Editor: Dirk M Elston, MD Share Email Print Feedback Close Sections Sections Paraneoplastic Pemphigus Treatment Medical Care Response to treatment paraneoplastic pemphigus is generally poor, especially for mucosal lesions. Initial care is aimed at treating superinfection, if present. Warm compresses, nonadherent wound dressings, and topical antibiotic ointment are helpful. Potent immunosuppressive agents

2014 eMedicine.com

46. Pemphigus, Paraneoplastic (Follow-up)

Pemphigus, Paraneoplastic (Follow-up) Paraneoplastic Pemphigus Treatment & Management: Medical Care, Surgical Care, Consultations Edition: No Results No Results Please confirm that you would like to log out of Medscape. If you log out, you will be required to enter your username and password the next time you visit. https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile&urlCache=aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvMTA2NDQ1Mi10cmVhdG1lbnQ= processing (...) > Paraneoplastic Pemphigus Treatment & Management Updated: Feb 07, 2019 Author: Lynne J Goldberg, MD; Chief Editor: Dirk M Elston, MD Share Email Print Feedback Close Sections Sections Paraneoplastic Pemphigus Treatment Medical Care Response to treatment paraneoplastic pemphigus is generally poor, especially for mucosal lesions. Initial care is aimed at treating superinfection, if present. Warm compresses, nonadherent wound dressings, and topical antibiotic ointment are helpful. Potent immunosuppressive agents

2014 eMedicine.com

47. Pemphigus, Paraneoplastic (Diagnosis)

Pemphigus, Paraneoplastic (Diagnosis) Paraneoplastic Pemphigus: Background, Pathophysiology, Etiology Edition: No Results No Results Please confirm that you would like to log out of Medscape. If you log out, you will be required to enter your username and password the next time you visit. https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile&urlCache=aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvMTA2NDQ1Mi1vdmVydmlldw== processing > Paraneoplastic Pemphigus (...) Updated: Feb 07, 2019 Author: Lynne J Goldberg, MD; Chief Editor: Dirk M Elston, MD Share Email Print Feedback Close Sections Sections Paraneoplastic Pemphigus Overview Background Anhalt et al [ ] first described paraneoplastic pemphigus in 1990. The authors reported five patients with underlying neoplasms who developed oral erosions and bullous skin eruptions. Skin biopsy samples showed both suprabasal acantholysis and interface dermatitis. Direct immunofluorescence (DIF) testing and indirect

2014 eMedicine.com

48. Pemphigus vulgaris as the first manifestation of multiple myeloma: a case report (PubMed)

to an immediate decline in monoclonal immunoglobulin G concentration. Skin and hematologic remission were maintained for 12 months.Absence of mucosal involvement, lack of vacuolar degeneration at the interface, and absence of apoptotic, dyskeratotic keratinocytes ruled out paraneoplastic pemphigus in our case. Pemphigus vulgaris should be considered even if possible underlying disease for which paraneoplastic pemphigus is recognized is present. (...) Pemphigus vulgaris as the first manifestation of multiple myeloma: a case report The association between pemphigus and malignancy has been well documented for decades but an association between pemphigus vulgaris and multiple myeloma is unusual. We report a case of pemphigus vulgaris revealing multiple myeloma.A 55-year-old Malagasy man, with no significant past medical history, presented with bullous and erosive skin lesions involving his trunk and scalp for the last 2 months. He had no mucous

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2018 Journal of medical case reports

49. A Typical Case of Paraneoplastic Pemphigus Without Detection of Malignancy: Effectiveness of Plasma Exchange. (PubMed)

A Typical Case of Paraneoplastic Pemphigus Without Detection of Malignancy: Effectiveness of Plasma Exchange. 24162880 2014 12 29 2014 05 07 1651-2057 94 3 2014 May Acta dermato-venereologica Acta Derm. Venereol. A typical case of paraneoplastic pemphigus without detection of malignancy: effectiveness of plasma exchange. 359-61 10.2340/00015555-1742 Kitagawa Chie C Department of Dermatology, Kochi Medical School, Kochi University, Kohasu, Okohcho, Nankoku, Kochi, 783-8505, Japan. jm-kitagawa (...) @kochi-u.ac.jp. Nakajima Kimiko K Aoyama Yumi Y Fujioka Ai A Nakajima Hideki H Tarutani Masahito M Tsuruta Daisuke D Hashimoto Takashi T Sano Shigetoshi S eng Case Reports Journal Article Sweden Acta Derm Venereol 0370310 0001-5555 IM Aged Biopsy Bronchiolitis Obliterans immunology therapy Disease Progression Fatal Outcome Female Humans Paraneoplastic Syndromes diagnosis immunology therapy Pemphigus diagnosis immunology therapy Plasma Exchange Time Factors Treatment Outcome 2013 10 29 6 0 2013 10 29

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2013 Acta Dermato-Venereologica

50. IgA Paraneoplastic Pemphigus in Angioimmunoblastic T-cell Lymphoma with Antibodies to Desmocollin 1, Type VII Collagen and Laminin 332. (PubMed)

IgA Paraneoplastic Pemphigus in Angioimmunoblastic T-cell Lymphoma with Antibodies to Desmocollin 1, Type VII Collagen and Laminin 332. 23824361 2014 09 11 2014 02 26 1651-2057 94 2 2014 Mar Acta dermato-venereologica Acta Derm. Venereol. IgA paraneoplastic pemphigus in angioimmunoblastic T-cell lymphoma with antibodies to desmocollin 1, type VII collagen and laminin 332. 235-6 10.2340/00015555-1660 Yashiro Mika M Department of Dermatology, Kitasato University School of Medicine, 1-15-1 (...) Collagen Type VII immunology Desmocollins immunology Humans Immunoblastic Lymphadenopathy blood immunology Immunoblotting Immunoglobulin A analysis immunology Immunoglobulin G immunology Lymphoma, T-Cell blood immunology Male Paraneoplastic Syndromes blood immunology pathology Pemphigus immunology pathology 2013 7 5 6 0 2013 7 5 6 0 2014 9 12 6 0 ppublish 23824361 10.2340/00015555-1660

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2013 Acta Dermato-Venereologica

51. Laboratory diagnosis of paraneoplastic pemphigus. (PubMed)

Laboratory diagnosis of paraneoplastic pemphigus. Paraneoplastic pemphigus (PNP) is a multiorgan disease characterized by antibodies against plakins, desmogleins and the α2-macroglobulin-like-1 (A2ML1) protein, in association with an underlying neoplasm. Accurate diagnosis relies on the demonstration of these autoantibodies in serum.To evaluate the value of different laboratory techniques in the serological diagnosis of PNP.We performed immunoblotting, envoplakin (EP) enzyme-linked

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2013 British Journal of Dermatology

52. Anti-Alpha-2-Macroglobulin-Like-1 Autoantibodies Are Detected Frequently and May Be Pathogenic in Paraneoplastic Pemphigus. (PubMed)

Anti-Alpha-2-Macroglobulin-Like-1 Autoantibodies Are Detected Frequently and May Be Pathogenic in Paraneoplastic Pemphigus. Paraneoplastic pemphigus (PNP) shows autoantibodies mainly to plakin and desmosomal cadherin family proteins. We have recently identified alpha-2-macroglobulin-like-1 (A2ML1), a broad range protease inhibitor, as a unique PNP antigen. In this study, we tested a large number of PNP sera by various methods. Forty (69.0%) of 58 PNP sera recognized A2ML1 recombinant protein

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2013 Journal of Investigative Dermatology

53. Routine detection of serum antidesmocollin autoantibodies is only useful in patients with atypical pemphigus. (PubMed)

against desmogleins 1 and 3 (n = 24) and sera from patients with autoimmune blistering diseases with positive direct IF microscopy taken at the time of diagnosis (n = 749). We found that detection of anti-Dsc serum reactivity is not helpful in the routine diagnosis of PV, pemphigus foliaceus and paraneoplastic pemphigus but may be valuable in pemphigus vegetans.© 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd. (...) Routine detection of serum antidesmocollin autoantibodies is only useful in patients with atypical pemphigus. Autoantibodies against the 3 desmocollin (Dsc; Dsc1-Dsc3) isoforms have been described in different pemphigus variants. Here, we developed state-of-the-art detection systems for serum anti-Dsc1, Dsc2 and Dsc1 IgG and IgA. These assays were applied in 5 different cohorts including pemphigus vulgaris (PV) patients with compatible direct immunofluorescence (IF) microscopy but no reactivity

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2017 Experimental Dermatology

54. Polyclonal Regulatory T Cells (PolyTregs) for Pemphigus

months prior to screening; Change in dosing frequency, concentration, or applied surface area of topical steroids and/or topical calcineurin inhibitors within 2 weeks prior to screening; Paraneoplastic pemphigus; Pemphigus erythematosus; Pemphigus vegetans; Immunoglobulin A (IgA) pemphigus; Drug-induced pemphigus; Blood donation within 10 weeks prior to baseline visit (Day 0); Hemoglobin < 10 g/dL; White blood cell (WBC) count < 3,000/ mm^3 (equivalent to < 3 x10^9/L); Lymphocyte count < 800/mm^3 (...) Polyclonal Regulatory T Cells (PolyTregs) for Pemphigus Polyclonal Regulatory T Cells (PolyTregs) for Pemphigus - Full Text View - ClinicalTrials.gov Hide glossary Glossary Study record managers: refer to the if submitting registration or results information. Search for terms x × Study Record Detail Saved Studies Save this study Warning You have reached the maximum number of saved studies (100). Please remove one or more studies before adding more. Polyclonal Regulatory T Cells (PolyTregs

2017 Clinical Trials

55. A Study to Evaluate the Safety, PD, PK and Efficacy of ARGX-113 in Patients With Pemphigus

that do not intend to use effective methods of contraception during the study or within 90 days after the last dosing. Confirmed diagnosis of paraneoplastic pemphigus, drug-induced pemphigus or any other non-PV/non-PF autoimmune blistering disease. History of refractory disease to active third line therapy (e.g. intravenous polyvalent human immunoglobulins [IVIg], rituximab, plasma exchange/ immunoadsorption). Use of therapies other than oral prednisone and conventional immunosuppressants, that can (...) A Study to Evaluate the Safety, PD, PK and Efficacy of ARGX-113 in Patients With Pemphigus A Study to Evaluate the Safety, PD, PK and Efficacy of ARGX-113 in Patients With Pemphigus - Full Text View - ClinicalTrials.gov Hide glossary Glossary Study record managers: refer to the if submitting registration or results information. Search for terms x × Study Record Detail Saved Studies Save this study Warning You have reached the maximum number of saved studies (100). Please remove one or more

2017 Clinical Trials

56. Analysis of Serum Cytokine Profile in Pemphigus (PubMed)

Analysis of Serum Cytokine Profile in Pemphigus Pemphigus is a group of autoimmune blistering diseases affecting skin and mucous membranes. While pemphigus is an autoantibody mediated disease, the role of T cells and cytokines in the pathogenesis is being increasingly recognized.This study was conducted to observe alterations in the serum cytokine levels of patients with pemphigus vulgaris (PV), pemphigus foliaceous (PF), paraneoplastic pemphigus (PNP) and compare with bullous pemphigoid (BP

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2017 Annals of dermatology

57. Diagnosis and classification of pemphigus and bullous pemphigoid. (PubMed)

Diagnosis and classification of pemphigus and bullous pemphigoid. Pemphigus and bullous pemphigoid represent the two major groups of autoimmune blistering diseases. Pemphigus has three major variants: pemphigus vulgaris, pemphigus foliaceus and paraneoplastic pemphigus and is characterized by autoantibodies directed against the cell surface of keratinocytes, producing acantholysis that in turn leads to intraepithelial blisters in the skin and/or mucous membranes. In bullous pemphigoid (...) , the autoantibodies are present at the dermo-epidermal junction and attack the hemidesmosomes, causing subepidermal blister formation. The classification of the major variants of both the pemphigus group and bullous pemphigoid can be based on the combination of clinical, histopathological and immunopathological criteria. Many tools are available for the diagnosis of these entities including biopsy, direct and indirect immunofluorescence, immunoprecipitation, immunoblotting and ELISA. However, currently

2017 Autoimmunity reviews

58. Pathogenic Anti-Desmoglein 3 mAbs Cloned from a Paraneoplastic Pemphigus Patient by Phage Display. (PubMed)

Pathogenic Anti-Desmoglein 3 mAbs Cloned from a Paraneoplastic Pemphigus Patient by Phage Display. Paraneoplastic pemphigus (PNP) is an autoimmune blistering disease associated with lymphoproliferative neoplasms and characterized by antibodies against plakins and desmoglein 3 (Dsg3). Anti-Dsg3 antibodies have a primary role in blister formation in PNP. In this study, we used phage display to clone monoclonal anti-Dsg3 antibodies from a PNP patient to further characterize their pathogenicity. We (...) . Epitope mapping using domain-swapped Dsg3/Dsg2 showed that these pathogenic mAbs bound Ca(2+)-dependent conformational epitopes in the middle portion of the extracellular region of Dsg3 (EC2 and EC3 domains), in contrast to most previously characterized pathogenic pemphigus vulgaris antibodies, which bound to the EC1 domain of Dsg3. These mAbs reflect the unique polyclonal nature of anti-Dsg3 antibodies in PNP and represent an important tool for detailing the pathophysiological mechanisms of blister

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2012 Journal of Investigative Dermatology

59. Paraneoplastic Pemphigus

Paraneoplastic Pemphigus Paraneoplastic Pemphigus Toggle navigation Brain Head & Neck Chest Endocrine Abdomen Musculoskeletal Skin Infectious Disease Hematology & Oncology Cohorts Diagnostics Emergency Findings Procedures Prevention & Management Pharmacy Resuscitation Trauma Emergency Procedures Ultrasound Cardiovascular Emergencies Lung Emergencies Infectious Disease Pediatrics Neurologic Emergencies Skin Exposure Miscellaneous Abuse Cancer Administration 4 Paraneoplastic Pemphigus (...) Paraneoplastic Pemphigus Aka: Paraneoplastic Pemphigus , Neoplasia-Associated Pemphigus From Related Chapters II. Epidemiology : Very rare Gender predominence: Female to male ratio of 2:1 Age of onset: 60 years III. Pathophysiology Autoantibodies to Desmoplakin 1 and 3 Lymphoid malignancy associated Thymoma Castleman's Disease IV. Signs Diffuse, painful bullous lesions Severe mucosal erosions in oropharynx and Polymorphic target-like skin lesions V. Differential Diagnosis VI. Lab Histology Suprabasilar

2015 FP Notebook

60. High-dose-rate intraluminal brachytherapy for paraneoplastic autoimmune multiorgan syndrome (PubMed)

High-dose-rate intraluminal brachytherapy for paraneoplastic autoimmune multiorgan syndrome Paraneoplastic autoimmune multiorgan syndrome (PAMS), also known as paraneoplasic pemphigus, involves the skin, internal organs and mucosa. PAMS-associated mortality may occur as a result of autoantibody formation against internal tumors and their infiltration into organs other than the skin lesions that characterize PAMS. The most common symptoms of PAMS include pain associated with continuous oral

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2016 Experimental and therapeutic medicine

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