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Abstract
AbstractParaneoplastic pemphigus is a rare and severe autoimmune blistering disease characterized by mucocutaneous lesions associated with benign and malignant neoplasms. Diagnostic criteria include the presence of chronic mucositis and polymorphic cutaneous lesions with occult or confirmed neoplasia; histopathological analysis exhibiting intraepidermal acantholysis, necrotic keratinocytes, and vacuolar interface dermatitis; direct immunofluorescence with intercellular deposits (IgG and C3) and at the basement membrane zone (IgG); indirect immunofluorescence with intercellular deposition of IgG (substrates: monkey esophagus and simple, columnar, and transitional epithelium); and, autoreactivity to desmogleins 1 and 3, desmocollins 1, 2, and 3, desmoplakins I and II, envoplakin, periplakin, epiplakin, plectin, BP230, and a-2-macroglobulin-like protein 1. Neoplasias frequently related to paraneoplastic pemphigus include chronic lymphocytic leukemia, non-Hodgkin lymphoma, carcinomas, Castleman disease, thymoma, and others. Currently, there is no standardized treatment for paraneoplastic pemphigus. Systemic corticosteroids, azathioprine, mycophenolate mofetil, cyclosporine, rituximab, cyclophosphamide, plasmapheresis, and intravenous immunoglobulin have been used, with variable outcomes. Reported survival rates in 1, 2, and 5 years are 49%, 41%, and 38%, respectively.
Highlights
Paraneoplastic pemphigus (PNP) is a rare autoimmune blistering disease characterized by polymorphous cutaneous lesions and chronic and recalcitrant mucositis, first reported by Anhalt et al.[1]
Further studies reported the role of potential antigenic targets in the pathogenesis of PNP: envoplakin, periplakin, desmoplakin I, desmoplakin II, epiplakin, plectin, BP230, desmoglein 1 (Dsg1), desmoglein 3 (Dsg3), desmocollin 1 (Dsc1), desmocollin 2 (Dsc2), desmocollin 3 (Dsc3), and α-2-macroglobulin-like protein 1 (A2ML1).[2,3,4,5,6,7,8,9]
The terms PNP and paraneoplastic autoimmune multiorgan syndrome” (PAMS) have been discussed in the literature; due to the pemphigus resemblance of the pathogenic antibody-mediated role of the disease, the term PNP is preferred.[10,11,12,13,14,15]
Summary
Paraneoplastic pemphigus (PNP) is a rare autoimmune blistering disease characterized by polymorphous cutaneous lesions and chronic and recalcitrant mucositis, first reported by Anhalt et al.[1]. The variable clinical presentations of PNP may be related to the predominant pathogenic mechanisms involved: antibody-mediated immunity - PNP lesions resembling pemphigus vulgaris and bullous pemphigoid; cell-mediated cytotoxicity - PNP lesions resembling erythema multiforme, graft-versus-host disease, and lichen planus. Multi-disciplinary mucosal evaluation Persistent oral erosions involving the lateral border of the tongue and extending to the vermillion of the lips are the most common manifestations of PNP; they represent the exclusive clinical presentation in 27% of patients.[1,32,40,43] Lesions may affect the nasopharynx, oropharynx, and larynx, leading to odynophagia, dysphagia, and hoarseness that require assessment by an ear, nose, and throat specialist. Plakin family Plakin family; higher diagnostic sensitivity[59] Plakin family; higher diagnostic sensitivity[59] Higher diagnostic sensitivity;[59] early PNP onset and lack of ocular involvement[8]
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