دورية أكاديمية
Paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome: Part I. Clinical overview and pathophysiology.
| العنوان: | Paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome: Part I. Clinical overview and pathophysiology. |
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| المؤلفون: | Anderson HJ; Department of Dermatology, Thomas Jefferson University, Philadelphia, Pennsylvania., Huang S; Department of Dermatology, Thomas Jefferson University, Philadelphia, Pennsylvania., Lee JB; Department of Dermatology, Thomas Jefferson University, Philadelphia, Pennsylvania. Electronic address: jason.lee@jefferson.edu. |
| المصدر: | Journal of the American Academy of Dermatology [J Am Acad Dermatol] 2024 Jul; Vol. 91 (1), pp. 1-10. Date of Electronic Publication: 2023 Aug 17. |
| نوع المنشور: | Journal Article; Review |
| اللغة: | English |
| بيانات الدورية: | Publisher: Mosby Country of Publication: United States NLM ID: 7907132 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1097-6787 (Electronic) Linking ISSN: 01909622 NLM ISO Abbreviation: J Am Acad Dermatol Subsets: MEDLINE |
| أسماء مطبوعة: | Original Publication: St. Louis, Mo., Mosby |
| مواضيع طبية MeSH: | Pemphigus*/immunology , Pemphigus*/diagnosis , Pemphigus*/physiopathology , Pemphigus*/etiology , Paraneoplastic Syndromes*/immunology , Paraneoplastic Syndromes*/diagnosis , Paraneoplastic Syndromes*/etiology , Paraneoplastic Syndromes*/physiopathology, Humans ; Bronchiolitis Obliterans/immunology ; Bronchiolitis Obliterans/etiology ; Bronchiolitis Obliterans/diagnosis ; Bronchiolitis Obliterans/physiopathology ; Autoimmune Diseases/immunology ; Autoimmune Diseases/complications ; Autoimmune Diseases/diagnosis ; Autoimmune Diseases/physiopathology ; Multiple Organ Failure/etiology ; Multiple Organ Failure/immunology ; Multiple Organ Failure/physiopathology ; Multiple Organ Failure/diagnosis ; Male ; Autoantibodies/immunology ; Autoantibodies/blood ; Female ; Risk Factors |
| مستخلص: | Paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome (PNP/PAMS) is a highly fatal autoimmune blistering disease. The condition occurs in patients with underlying benign or malignant neoplasms, most commonly lymphoproliferative disorders. Both humoral and cell-mediated immunities contribute to the pathogenesis, and autoantibodies against plakin family proteins are characteristic. Patients typically present with severe stomatitis and polymorphous skin lesions, which are often resistant to treatment. Bronchiolitis obliterans (BO) is a frequent complication which contributes to the high mortality rate of PNP/PAMS. Given the rarity of this disorder and heterogeneity of clinical presentation, clinicians should maintain a high index of suspicion for PNP/PAMS to avoid delayed diagnosis. In this first part of a two-part continuing medical education (CME) series, risk factors, pathogenesis, and clinical features of PNP/PAMS are discussed. Competing Interests: Conflicts of interest None disclosed. (Copyright © 2023 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.) |
| فهرسة مساهمة: | Keywords: CLL; Castleman disease; bronchiolitis obliterans; non-Hodgkin lymphoma; paraneoplastic; paraneoplastic autoimmune multiorgan syndrome; paraneoplastic pemphigus; pemphigus; plakin |
| المشرفين على المادة: | 0 (Autoantibodies) |
| تواريخ الأحداث: | Date Created: 20230819 Date Completed: 20240621 Latest Revision: 20240621 |
| رمز التحديث: | 20240622 |
| DOI: | 10.1016/j.jaad.2023.08.020 |
| PMID: | 37597771 |
| قاعدة البيانات: | MEDLINE |
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Full Text Finder | تدمد: | 1097-6787 |
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| DOI: | 10.1016/j.jaad.2023.08.020 |