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Paraneoplastic Pemphigus

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21. Paraneoplastic pemphigus: a short review (PubMed)

Paraneoplastic pemphigus: a short review Paraneoplastic pemphigus (PNP) is a fatal autoimmune blistering disease associated with an underlying malignancy. It is a newly recognized blistering disease, which was first recognized in 1990 by Dr Anhalt who described an atypical pemphigus with associated neoplasia. In 2001, Nguyen proposed the term paraneoplastic autoimmune multiorgan syndrome because of the recognition that the condition affects multiple organ systems. PNP presents most frequently (...) for systemic complications (to identify tumor), skin biopsies (for histopathological and immunofluorescence studies), and serum immunological studies. PNP is characterized by the presence of autoantibodies against antigens such as desmoplakin I (250 kD), bullous pemphigoid aniygen I (230 kD), desmoplakin II (210 kD), envoplakin (210 kD), periplakin (190 kD), plectin (500 kD), and a 170 kD protein. Unlike other forms of pemphigus, PNP can affect other types of epithelia, such as gastrointestinal

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2016 Clinical, cosmetic and investigational dermatology

22. Paraneoplastic Pemphigus and Autoimmune Blistering Diseases Associated with Neoplasm: Characteristics, Diagnosis, Associated Neoplasms, Proposed Pathogenesis, Treatment. (PubMed)

Paraneoplastic Pemphigus and Autoimmune Blistering Diseases Associated with Neoplasm: Characteristics, Diagnosis, Associated Neoplasms, Proposed Pathogenesis, Treatment. Autoimmune paraneoplastic and neoplasm-associated skin syndromes are characterized by autoimmune-mediated cutaneous lesions in the presence of a neoplasm. The identification of these syndromes provides information about the underlying tumor, systemic symptoms, and debilitating complications. The recognition of these syndromes (...) diagnostic criteria, current understanding of pathogenesis, and treatment options for a selection of four diseases are reviewed. Paraneoplastic pemphigus manifests with clinically distinct painful mucosal erosions and polymorphic cutaneous lesions, and is often associated with lymphoproliferative neoplasm. In contrast, bullous pemphigoid associated with neoplasm presents with large tense subepidermal bullae of the skin, and mild mucosal involvement, but without unique clinical features. Mucous membrane

2016 American journal of clinical dermatology

23. Epiplakin Is a Paraneoplastic Pemphigus Autoantigen and Related to Bronchiolitis Obliterans in Japanese Patients. (PubMed)

Epiplakin Is a Paraneoplastic Pemphigus Autoantigen and Related to Bronchiolitis Obliterans in Japanese Patients. All plakin family proteins are known to be autoantigens in paraneoplastic pemphigus (PNP). In this study, we first examined whether PNP sera also react with epiplakin, another plakin protein, by various immunological methods using 48 Japanese PNP sera. Immunofluorescence confirmed that cultured keratinocytes expressed epiplakin. Epiplakin was detected by 72.9% of PNP sera

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2016 Journal of Investigative Dermatology

24. Inflammatory myofibroblastic tumor presenting as paraneoplastic pemphigus in a 7-year-old girl (PubMed)

Inflammatory myofibroblastic tumor presenting as paraneoplastic pemphigus in a 7-year-old girl 27051823 2016 04 07 2019 02 26 2352-5126 2 1 2016 Jan JAAD case reports JAAD Case Rep Inflammatory myofibroblastic tumor presenting as paraneoplastic pemphigus in a 7-year-old girl. 37-40 10.1016/j.jdcr.2015.10.009 Halpert Evelyne E Department of Dermatology, Fundación Santafe de Bogotá, Bogotá, Colombia. Figueroa Juan Luis JL Department of Pediatrics, Fundación Santafe de Bogotá, Bogotá, Colombia (...) Bogotá, Bogotá, Colombia. Olmos Carlos C Department of Pediatrics, Fundación Santafe de Bogotá, Bogotá, Colombia. eng Case Reports 2016 01 26 United States JAAD Case Rep 101665210 2352-5126 myofibroblastic tumor paraneoplastic syndromes pediatrics pemphigus vulgaris 2016 4 7 6 0 2016 4 7 6 0 2016 4 7 6 1 epublish 27051823 10.1016/j.jdcr.2015.10.009 S2352-5126(15)00174-5 PMC4809448 J Thorac Cardiovasc Surg. 2003 Feb;125(2):400-6 12579111 Int J Dermatol. 2009 Feb;48(2):162-9 19200194 N Engl J Med. 1990

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2016 JAAD Case Reports

25. Castleman's Disease Presenting as Localized Abdominal Mass and Paraneoplastic Pemphigus (PubMed)

Castleman's Disease Presenting as Localized Abdominal Mass and Paraneoplastic Pemphigus Castleman's disease is a rare, benign lymphoproliferative disorder of unknown origin. Paraneoplastic pemphigus is a common association which presents as oral mucosal ulcerations. Abdominal and retroperitoneal Castleman's disease present either as a localized disease or as a systemic disease. We hereby present a 15-year-old male patient with oral mucosal lesions with localized vague right lower abdominal mass (...) who was diagnosed to have Castleman's disease with paraneoplastic pemphigus which was surgically excised.

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2016 Journal of clinical imaging science

26. Paraneoplastic Pemphigus Associated with Follicular Dendritic Cell Tumor in the Mediastinum (PubMed)

Paraneoplastic Pemphigus Associated with Follicular Dendritic Cell Tumor in the Mediastinum Paraneoplastic Pemphigus (PNP) is an autoimmune bullous disease characterized by severe stomatitis, polymorphous skin eruptions, and underlying neoplasms. Diagnosis of cutaneous paraneoplastic disorders requires high index of suspicion. We describe a patient with PNP associated with follicular dendritic cell (FDC) tumor in the mediastinum, a rare neoplasm originating from follicular dendritic cells. Its

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2016 Case reports in dermatological medicine

27. 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

2018 FP Notebook

28. Extremities of the N-terminus of envoplakin and C-terminus of its linker subdomain are major epitopes of paraneoplastic pemphigus. (PubMed)

Extremities of the N-terminus of envoplakin and C-terminus of its linker subdomain are major epitopes of paraneoplastic pemphigus. Autoantibodies against N-terminal domains and linker subdomains of envoplakin (EVPL) and periplakin (PPL) were frequently detected in sera of paraneoplastic pemphigus(PNP) patients.To further investigate finer epitopes of EVPL and PPL, and evaluate their associations with clinical aspects of PNP.We produced 12 overlapping truncated fragments of these regions

2016 Journal of dermatological science

29. Recurrent corneal melting in the paraneoplastic pemphigus associated with Castleman's disease. (PubMed)

Recurrent corneal melting in the paraneoplastic pemphigus associated with Castleman's disease. The ocular presentation of Castleman's disease (CD)-associated paraneoplastic pemphigus (PNP) has rarely been reported. In this report, we describe a young patient with CD-associated PNP who had recurrent corneal ulceration in addition to cicatrizing conjunctivitis.We describe a case of 23-year-old male with mucocutaneous erosion and conjunctival injection and erosion who was found to have PNP. Pelvic

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2016 BMC Ophthalmology

30. Paraneoplastic pemphigus with anti-BP180 autoantibodies and Castleman's disease. (PubMed)

Paraneoplastic pemphigus with anti-BP180 autoantibodies and Castleman's disease. 27436786 2018 06 25 2018 06 25 1365-2133 176 3 2017 03 The British journal of dermatology Br. J. Dermatol. Paraneoplastic pemphigus with anti-BP180 autoantibodies and Castleman disease. 824-826 10.1111/bjd.14877 Maier L L Department of Dermatology, Sozialmedizinisches Zentrum Ost Donauspital, Vienna, Austria. Medical Research Society Donaustadt, Vienna, Austria. Udvardi A A Department of Dermatology (...) Diseases immunology Mouth Mucosa immunology Non-Fibrillar Collagens immunology Paraneoplastic Syndromes immunology Pemphigus immunology 2016 7 21 6 0 2018 6 26 6 0 2016 7 21 6 0 ppublish 27436786 10.1111/bjd.14877

2016 British Journal of Dermatology

31. Peri-anal Paraneoplastic Pemphigus Heralding the Relapse of Follicular Lymphoma and Its Successful Management by Rituximab: A Short Correspondence (PubMed)

Peri-anal Paraneoplastic Pemphigus Heralding the Relapse of Follicular Lymphoma and Its Successful Management by Rituximab: A Short Correspondence 27812271 2018 11 13 0971-4502 32 4 2016 Dec Indian journal of hematology & blood transfusion : an official journal of Indian Society of Hematology and Blood Transfusion Indian J Hematol Blood Transfus Peri-anal Paraneoplastic Pemphigus Heralding the Relapse of Follicular Lymphoma and Its Successful Management by Rituximab: A Short Correspondence. 519

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2016 Indian Journal of Hematology & Blood Transfusion

32. A Case Report on Paraneoplastic Pemphigus Associated Colonic Carcinoma (PubMed)

A Case Report on Paraneoplastic Pemphigus Associated Colonic Carcinoma 27270550 2017 03 13 2018 11 13 2542-5641 129 12 2016 Jun 20 Chinese medical journal Chin. Med. J. A Case Report on Paraneoplastic Pemphigus Associated Colonic Carcinoma. 1501-2 10.4103/0366-6999.183422 Wang Yan-Zhao YZ Department of General Surgery, Peking University Shougang Hospital, Beijing 100144, China. Gao Zhao-Ya ZY Department of General Surgery, Peking University Shougang Hospital, Beijing 100144, China. Lei Fu-Ming (...) FM Department of General Surgery, Peking University Shougang Hospital, Beijing 100144, China. Zhang Jin-Xia JX Department of Pathology, Peking University Shougang Hospital, Beijing 100144, China. Gu Jin J Department of General Surgery, Peking University Shougang Hospital, Beijing 100144, China. eng Case Reports Journal Article China Chin Med J (Engl) 7513795 0366-6999 IM Aged Colonic Neoplasms diagnosis Humans Male Paraneoplastic Syndromes diagnosis Pemphigus diagnosis 2016 6 9 6 0 2016 6 9 6 0

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2016 Chinese medical journal

33. Pemphigus

Pemphigus Pemphigus - Symptoms, diagnosis and treatment | BMJ Best Practice You'll need a subscription to access all of BMJ Best Practice Search  Pemphigus Last reviewed: February 2019 Last updated: January 2019 Summary Group of autoimmune blistering diseases. Pemphigus foliaceus (PF) is confined to the skin. Pemphigus vulgaris (PV) and paraneoplastic pemphigus (PNP) can involve the skin and mucosal surfaces of the eyes, mouth, nasopharynx, and oesophagus. Diagnosis is based on clinical (...) non-Hodgkin's lymphoma, and mortality can approach 90% due to respiratory failure from bronchiolitis obliterans. Definition Pemphigus describes a group of autoimmune blistering diseases that involve the epidermal surfaces of the skin, mucosa, or both. There are 3 broad categories: pemphigus vulgaris (PV), pemphigus foliaceus (PF), and paraneoplastic pemphigus (PNP). Pemphigus is mediated by an autoantibody that binds a specific component of the desmosomal plaque. The desmosomal plaque holds

2018 BMJ Best Practice

34. Clinical and immunological findings in 104 cases of paraneoplastic pemphigus. (PubMed)

Clinical and immunological findings in 104 cases of paraneoplastic pemphigus. Although there are many reports of sporadic patients with paraneoplastic pemphigus (PNP), only a few systematic studies on large cohorts of patients with PNP have been reported.To analyse the clinical and immunological findings in a large cohort of patients with PNP.This retrospective study consisted of 104 patients with PNP. Clinical and histopathological manifestations, associated neoplasms, complicating diseases

2015 British Journal of Dermatology

35. Reply to: Clinical and immunological findings in 104 cases of paraneoplastic pemphigus. (PubMed)

Reply to: Clinical and immunological findings in 104 cases of paraneoplastic pemphigus. 26473404 2016 06 13 2018 12 02 1365-2133 174 2 2016 Feb The British journal of dermatology Br. J. Dermatol. Reply to: Clinical and immunological findings in 104 cases of paraneoplastic pemphigus. 461 10.1111/bjd.14234 Amber K T KT Department of Dermatology, University of California Irvine Medical Center, Irvine, CA, U.S.A.. KAmber@UCI.edu. eng Letter Comment 2015 12 27 England Br J Dermatol 0004041 0007-0963

2015 British Journal of Dermatology

36. Epiplakin is a Paraneoplastic Pemphigus Autoantigen and Related to Bronchiolitis Obliterans in Japanese Patients. (PubMed)

Epiplakin is a Paraneoplastic Pemphigus Autoantigen and Related to Bronchiolitis Obliterans in Japanese Patients. All plakin family proteins are known to be autoantigens in paraneoplastic pemphigus (PNP). In this study, we first examined whether PNP sera also react with epiplakin, another plakin protein, by various immunological methods using 48 Japanese PNP sera. Immunofluorescence confirmed that cultured keratinocytes expressed epiplakin. Epiplakin was detected by 72.9% of PNP sera

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2015 Journal of Investigative Dermatology

37. Direct and indirect immunofluorescence staining patterns in the diagnosis of paraneoplastic pemphigus. (PubMed)

Direct and indirect immunofluorescence staining patterns in the diagnosis of paraneoplastic pemphigus. 26556228 2017 03 06 2017 08 17 1365-2133 174 4 2016 Apr The British journal of dermatology Br. J. Dermatol. Direct and indirect immunofluorescence staining patterns in the diagnosis of paraneoplastic pemphigus. 912-5 10.1111/bjd.14282 Poot A M AM Center for Blistering Diseases, Department of Dermatology, University Medical Center Groningen, University of Groningen, Hanzeplein 1, 9700 RB (...) Center Groningen, University of Groningen, Hanzeplein 1, 9700 RB, Groningen, the Netherlands. Diercks G F H GF Center for Blistering Diseases, Department of Dermatology, University Medical Center Groningen, University of Groningen, Hanzeplein 1, 9700 RB, Groningen, the Netherlands. eng Letter 2016 01 27 England Br J Dermatol 0004041 0007-0963 0 Autoantigens IM Autoantigens metabolism Fluorescent Antibody Technique, Direct Fluorescent Antibody Technique, Indirect Humans Paraneoplastic Syndromes

2015 British Journal of Dermatology

38. Clinical and laboratory characterization of 114 cases of Castleman disease patients from a single centre: paraneoplastic pemphigus is an unfavourable prognostic factor. (PubMed)

Clinical and laboratory characterization of 114 cases of Castleman disease patients from a single centre: paraneoplastic pemphigus is an unfavourable prognostic factor. This study retrospectively collected the clinical and laboratory data of 114 patients with Castleman disease (CD) from a single medical centre. Clinical classification identified 62 patients (54·4%) with unicentric Castleman disease and 52 (45·6%) with multi-centric Castleman disease. Pathological classification revealed 68 (...) cases (59·6%) of hyaline vascular variant, 16 (14·1%) mixed cellular variant (Mix) and 30 (26·3%) plasmacytic variant. Clinical complications occurred in 69 CD patients, including 37 cases of paraneoplastic pemphigus (PNP) and 25 cases with renal complications. Haematological involvement, pleural effusion and/or ascites and POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes) were also found. Univariate analysis showed that presence of clinical

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2015 British journal of haematology

39. Paraneoplastic pemphigus with eosinophilic spongiosis and autoantibodies against desmocollins 2 and 3. (PubMed)

Paraneoplastic pemphigus with eosinophilic spongiosis and autoantibodies against desmocollins 2 and 3. Paraneoplastic pemphigus (PNP) is an autoimmune blistering disease associated with neoplasms, typically lymphoproliferative disorders. PNP is characterized clinically by painful erosive stomatitis and polymorphous skin lesions. Histopathological findings are also very varied, and include lichen planus-like and pemphigus-like changes. These polymorphic clinicopathological findings are probably (...) due to the complex pathogenic mechanism, in which both cellular and humoral immunity are implicated. Eosinophilic spongiosis, although infrequent, can be found with pemphigus herpetiformis and bullous pemphigoid, although this association has not been established in PNP. The presence of autoantibodies against envoplakin and periplakin in PNP has been reported, but autoantibodies against desmocollins (Dscs) have been found in only a very few cases of PNP, probably due to the lack of studies

2014 Clinical & Experimental Dermatology

40. Paraneoplastic pemphigus associated with metastatic lymphoepithelioma-like carcinoma originating from the thyroid gland. (PubMed)

Paraneoplastic pemphigus associated with metastatic lymphoepithelioma-like carcinoma originating from the thyroid gland. 25112893 2015 12 18 2015 03 17 1365-2133 172 3 2015 Mar The British journal of dermatology Br. J. Dermatol. Paraneoplastic pemphigus associated with metastatic lymphoepithelioma-like carcinoma originating from the thyroid gland. 831-4 10.1111/bjd.13334 Hong W J WJ Department of Dermatology and Cutaneous Biology Research Institute, Gangnam Severance Hospital, Yonsei University (...) College of Medicine, Seoul, South Korea. Lee S E SE Chang S-E SE Hashimoto T T Kim S-C SC eng Case Reports Letter 2015 01 28 England Br J Dermatol 0004041 0007-0963 IM Carcinoma complications Fatal Outcome Female Humans Middle Aged Neoplasm Metastasis Paraneoplastic Syndromes complications Pemphigus etiology Thyroid Neoplasms complications 2014 8 13 6 0 2014 8 13 6 0 2015 12 19 6 0 ppublish 25112893 10.1111/bjd.13334

2014 British Journal of Dermatology

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