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Paraneoplastic Pemphigus

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1. Paraneoplastic autoimmune multiorgan syndrome (PAMS): Beyond the single phenotype of paraneoplastic pemphigus. (PubMed)

Paraneoplastic autoimmune multiorgan syndrome (PAMS): Beyond the single phenotype of paraneoplastic pemphigus. Paraneoplastic autoimmune multiorgan syndrome (PAMS) is characterized by a heterogenous group of signs and symptoms including severe desquamative stomatitis, a polymorphous cutaneous eruption, humoral immunity against plakin proteins, contribution of cell-mediated autoimmunity and commonly a progressive respiratory failure. Autoantibodies in PAMS target a wide array of antigens (...) including plakins, cadherins, alpha-2-macroglobulin like 1 (A2ML1), BP180, plakophilin-3, and several neuromuscular antigens. Originally described as paraneoplastic pemphigus in 1990 due to some of its clinical and immunologic similarities to classic pemphigus (pemphigus vulgaris and pemphigus foliaceus), PAMS is a multiorganopathy with several distinct features from these classic forms of pemphigus. Epidemiologically, PAMS is associated with underlying neoplasia and has a differing HLA-II allele

2018 Autoimmunity reviews

2. Successful use of Bruton's kinase inhibitor, ibrutinib, to control paraneoplastic pemphigus in a patient with paraneoplastic autoimmune multiorgan syndrome and chronic lymphocytic leukaemia. (PubMed)

Successful use of Bruton's kinase inhibitor, ibrutinib, to control paraneoplastic pemphigus in a patient with paraneoplastic autoimmune multiorgan syndrome and chronic lymphocytic leukaemia. We present the case of a 51-year-old man who developed paraneoplastic pemphigus (PNP) in the context of chronic lymphocytic leukemia (CLL). His CLL was successfully controlled with ibrutinib. Concurrently, there was significant improvement of his PNP, suggesting that ibrutinib may be a very useful addition

2017 Australasian Journal of Dermatology

3. Risk factors for death and survival in paraneoplastic pemphigus associated with hematologic malignancies in adults. (PubMed)

Risk factors for death and survival in paraneoplastic pemphigus associated with hematologic malignancies in adults. Paraneoplastic pemphigus (PNP) occurs more often in patients with hematologic malignancies (HMs) than in patients with solid cancer. Lung bronchiolitis obliterans (BO) is a severe complication of PNP.To determine the precise clinical and biologic features of HM-associated PNP and identify factors associated with mortality and survival.Systematic review of previously described

2019 Journal of American Academy of Dermatology

4. Paraneoplastic Pemphigus as a First Manifestation of an Intra-Abdominal Follicular Dendritic Cell Sarcoma: Rare Case and Review of the Literature (PubMed)

Paraneoplastic Pemphigus as a First Manifestation of an Intra-Abdominal Follicular Dendritic Cell Sarcoma: Rare Case and Review of the Literature Follicular dendritic cell sarcoma (FDCS) is an extremely rare malignant tumor caused by proliferation of antigen-presenting follicular dendritic cells. The tumor most commonly arises in lymph nodes, with fewer than 30% of cases originating in extranodal sites.We report the case of a 39-year-old previously healthy male patient who presented (...) with paraneoplastic pemphigus in the setting of a large intraperitoneal mass with no associated lymphadenopathy. The biopsy results showed a cellular proliferation of mildly to moderately atypical oval-to-spindle-shaped cells admixed with a variably dense lymphoplasmacytic infiltrate suggestive of FDCS versus inflammatory myofibroblastic tumor. A pathology review following total resection of the abdominal mass confirmed the diagnosis of FDCS. The tumor cells showed positivity for CD35, CD21, CD45, and CD68

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2018 Case reports in oncology

5. Unicentric castleman disease complicated by paraneoplastic bronchiolitis obliterans and pemphigus (PubMed)

Unicentric castleman disease complicated by paraneoplastic bronchiolitis obliterans and pemphigus Bronchiolitis obliterans (BO) and paraneoplastic pemphigus are rare and ominous complications of Castleman disease. Collectively, these processes have been reported as part of paraneoplastic autoimmune multiorgan syndrome (PAMS), and they can occur in the setting of various hematologic malignant tumors, carcinoid tumors, and melanoma. Irrespective of the underlying malignancy driving PAMS (...) , the clinical outcomes are uniformly poor, and there are no standard treatment regimens, given the clinical rarity of the syndrome. We describe 2 patients with unicentric Castleman disease complicated by paraneoplastic pemphigus and bronchiolitis obliterans. In addition to primary surgical resection for Castleman disease, we also used therapy from a treatment protocol used for bronchiolitis obliterans resulting from hematopoietic stem cell transplant (HSCT). We were able to treat the patients using

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2018 Respiratory Medicine Case Reports

6. Paraneoplastic pemphigus associated with chronic lymphocytic leukemia: a case report (PubMed)

Paraneoplastic pemphigus associated with chronic lymphocytic leukemia: a case report Paraneoplastic pemphigus is a rare multiorgan disease of autoimmune causes, usually triggered by neoplasias, mainly of lymphoproliferative origin, such as leukemia and lymphoma. This disorder is categorized by the presence of autoantibodies that react against proteins, such as desmoplakins, desmogleins, desmocollins, and others that exist in cellular junctions. Paraneoplastic pemphigus can manifest clinically (...) in a variety of ways, ranging from mucositis to lesions involving the skin and pulmonary changes. The diagnosis depends on the correlation between the clinical and histopathologic evaluations. Currently, the treatment of this disease is still very difficult and ineffective. The prognosis is poor, and the mortality rate is very high.We report a case of a Caucasian patient who had chronic lymphocytic leukemia and developed paraneoplastic pemphigus with severe impairment of skin and mucosa. The initial

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2018 Journal of medical case reports

7. Paraneoplastic pemphigus with anti-desmocollin 3 autoantibodies and chronic lymphocytic leukemia (PubMed)

Paraneoplastic pemphigus with anti-desmocollin 3 autoantibodies and chronic lymphocytic leukemia 29599682 2018 11 14 1642-395X 35 1 2018 Feb Postepy dermatologii i alergologii Postepy Dermatol Alergol Paraneoplastic pemphigus with anti-desmocollin 3 autoantibodies and chronic lymphocytic leukemia. 113-115 10.5114/ada.2018.73171 Adaszewska Alicja A Department of Dermatology and Immunodermatology, Medical University of Warsaw, Warsaw, Poland. Ishii Norito N Department of Dermatology, Kurume

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2018 Advances in Dermatology and Allergology/Postȩpy Dermatologii i Alergologii

8. Paraneoplastic Pemphigus Associated with B-cell Chronic Lymphocytic Leukemia Treated with Ibrutinib and Rituximab (PubMed)

Paraneoplastic Pemphigus Associated with B-cell Chronic Lymphocytic Leukemia Treated with Ibrutinib and Rituximab Paraneoplastic pemphigus (PNP) is a severe autoimmune blistering disease associated with an underlying malignancy, and its prognosis is poor. We herein report the first patient with B-cell chronic lymphocytic leukemia/small lymphocytic lymphoma (B-CLL/SLL)-associated PNP successfully treated with the Bruton's tyrosine kinase inhibitor ibrutinib and rituximab. Although his PNP

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2018 Internal Medicine

9. Paraneoplastic pemphigus in two pairs of brothers. (PubMed)

Paraneoplastic pemphigus in two pairs of brothers. 28622444 2017 12 27 1468-3083 31 12 2017 Dec Journal of the European Academy of Dermatology and Venereology : JEADV J Eur Acad Dermatol Venereol Paraneoplastic pemphigus in two pairs of brothers. e552-e553 10.1111/jdv.14418 Kranzelbinder B B Department of Dermatology, Medical University of Graz, Graz, Austria. Hashimoto T T Department of Dermatology, Kurume University School of Medicine, Fukuoka, Japan. Joch M M Department of Dermatology

2017 Journal of the European Academy of Dermatology and Venereology

10. Paraneoplastic pemphigus seen in 4 patients with hematologic malignancies formerly treated with rituximab. (PubMed)

Paraneoplastic pemphigus seen in 4 patients with hematologic malignancies formerly treated with rituximab. 28758262 2018 02 21 1468-3083 32 2 2018 Feb Journal of the European Academy of Dermatology and Venereology : JEADV J Eur Acad Dermatol Venereol Paraneoplastic pemphigus seen in four patients with haematological malignancies formerly treated with rituximab. e50-e52 10.1111/jdv.14500 Baykal C C Department of Dermatology and Venereology, Istanbul Medical Faculty, Istanbul University, Istanbul

2017 Journal of the European Academy of Dermatology and Venereology

11. Paraneoplastic pemphigus associated with chronic lymphocytic leukemia: A case report. (PubMed)

Paraneoplastic pemphigus associated with chronic lymphocytic leukemia: A case report. Paraneoplastic pemphigus (PNP) is an autoimmune syndrome associated with neoplasms. The treatment approach principally includes suppressing the immunity, but its therapeutic effect is not satisfying.We report a case of paraneoplastic pemphigus linked to chronic lymphocytic leukemia in a 63-year-old man.At first, the patient was diagnosed with pityriasis rose caused by a viral infection. Biopsies for histology

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2017 Medicine

12. Paraneoplastic pemphigus associated with Castleman disease: Progression from mucous to mucocutaneous lesions with epitope-spreading phenomena. (PubMed)

Paraneoplastic pemphigus associated with Castleman disease: Progression from mucous to mucocutaneous lesions with epitope-spreading phenomena. 28213962 2018 07 09 2018 07 09 1365-2133 176 5 2017 05 The British journal of dermatology Br. J. Dermatol. Paraneoplastic pemphigus associated with Castleman disease: progression from mucous to mucocutaneous lesions with epitope-spreading phenomena. 1406-1409 10.1111/bjd.15389 Okahashi K K Department of Dermatology, Kindai University Faculty of Medicine (...) , Kurume, Japan. Kawada A A Department of Dermatology, Kindai University Faculty of Medicine, Osaka-Sayama, Japan. eng Case Reports Letter 2017 03 22 England Br J Dermatol 0004041 0007-0963 0 Epitopes IM Castleman Disease complications Disease Progression Epitopes physiology Fatal Outcome Humans Male Middle Aged Mouth Diseases complications Paraneoplastic Syndromes complications Pemphigus complications 2017 2 19 6 0 2018 7 10 6 0 2017 2 19 6 0 ppublish 28213962 10.1111/bjd.15389

2017 British Journal of Dermatology

13. Paraneoplastic pemphigus mimicking toxic epidermal necrolysis: An underdiagnosed entity? (PubMed)

Paraneoplastic pemphigus mimicking toxic epidermal necrolysis: An underdiagnosed entity? 29387753 2019 02 26 2352-5126 4 1 2018 Jan JAAD case reports JAAD Case Rep Paraneoplastic pemphigus mimicking toxic epidermal necrolysis: An underdiagnosed entity? 67-71 10.1016/j.jdcr.2017.11.002 McLarney R Matthew RM Department of Dermatology, Temple University Lewis Katz School of Medicine, Philadelphia, Pennsylvania. Valdes-Rodriguez Rodrigo H RH Department of Dermatology, Temple University Lewis Katz (...) of Medicine, Gainesville, Florida. eng Case Reports 2017 12 19 United States JAAD Case Rep 101665210 2352-5126 CLL, chronic lymphocytic leukemia DSG, desmoglein ELISA, enzyme-linked immunosorbent assay EP, envoplakin IIF, indirect immunofluorescence PNP, paraneoplastic pemphigus PP, periplakin SJS, Stevens-Johnson syndrome Stevens-Johnson syndrome TEN, toxic epidermal necrolysis paraneoplastic pemphigus toxic epidermal necrolysis 2018 2 2 6 0 2018 2 2 6 0 2018 2 2 6 1 epublish 29387753 10.1016/j.jdcr

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2017 JAAD Case Reports

14. Paraneoplastic Pemphigus: Insight into the Autoimmune Pathogenesis, Clinical Features and Therapy (PubMed)

Paraneoplastic Pemphigus: Insight into the Autoimmune Pathogenesis, Clinical Features and Therapy Paraneoplastic pemphigus is a rare autoimmune skin disease that is always associated with a neoplasm. Usually, oral, skin, and mucosal lesions are the earliest manifestations shown by paraneoplastic pemphigus patients. The pathogenesis of paraneoplastic pemphigus is not yet completely understood, although some immunological aspects have been recently clarified. Because of its rarity, several (...) diagnostic criteria have been proposed. Besides, several diagnostic procedures have been used for the diagnosis, including indirect immunofluorescence, direct immunofluorescence, and ELISA. We reviewed the most recent literature, searching on PubMed "paraneoplastic pemphigus". We included also papers in French, German, and Spanish. We found 613 papers for "paraneoplastic pemphigus". Among them, 169 were review papers. Because of its varying clinical features, paraneoplastic pemphigus still represents

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2017 International journal of molecular sciences

15. Atypical laboratory presentation of paraneoplastic pemphigus associated with Castleman disease (PubMed)

Atypical laboratory presentation of paraneoplastic pemphigus associated with Castleman disease 28367488 2019 02 26 2352-5126 3 2 2017 Mar JAAD case reports JAAD Case Rep Atypical laboratory presentation of paraneoplastic pemphigus associated with Castleman disease. 138-139 10.1016/j.jdcr.2017.01.016 Lonowski Sarah S David Geffen School of Medicine, University of California Los Angeles, Los Angeles, California. Goldbach Hayley H Department of Medicine, Division of Dermatology, University (...) of California Los Angeles, Los Angeles, California. Holland Vanessa V Department of Medicine, Division of Dermatology, University of California Los Angeles, Los Angeles, California. eng Case Reports 2017 03 15 United States JAAD Case Rep 101665210 2352-5126 Castleman disease ELISA, enzyme-linked immunosorbent assay IIF, indirect Immunofluorescence PNP, paraneoplastic pemphigus PV, pemphigus vulgaris autoimmune skin disease bullous disease direct and indirect immunofluorescence paraneoplastic pemphigus

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2017 JAAD Case Reports

16. Paraneoplastic Pemphigus Associated with a Malignant Thymoma: A Case of Persistent and Refractory Oral Ulcerations Following Thymectomy (PubMed)

Paraneoplastic Pemphigus Associated with a Malignant Thymoma: A Case of Persistent and Refractory Oral Ulcerations Following Thymectomy Paraneoplastic pemphigus is a rare, life-threatening autoimmune mucocutaneous blistering disease associated with underlying neoplasia, commonly lymphoproliferative tumors. Herein we report a case of paraneoplastic pemphigus with a unique autoantibody profile associated with a malignant thymoma. A 56-year-old female patient presented with relapsing oral

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2017 Annals of dermatology

17. 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

2019 BMJ Best Practice

18. Paraneoplastic pemphigus and myasthenia gravis, associated with inflammatory pseudotumor‐like follicular dendritic cell sarcoma: response to rituximab (PubMed)

Paraneoplastic pemphigus and myasthenia gravis, associated with inflammatory pseudotumor‐like follicular dendritic cell sarcoma: response to rituximab Paraneoplastic pemphigus (PNP) is an autoimmune blistering disease associated with neoplasms. The disease is most commonly of lymphoproliferative origin and presents high mortality. We describe a patient with PNP and myasthenia gravis associated with inflammatory pseudotumor-like follicular dendritic cell sarcoma, as well as the response

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2016 Clinical Case Reports

19. 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

20. 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

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