Paraneoplastic Pemphigus: Insight into the Autoimmune Pathogenesis, Clinical Features and Therapy

Paolino, Giovanni; Didona, Dario; Magliulo, Giuseppe; Iannella, Giannicola; Didona, Biagio; Mercuri, Santo Raffaele; Moliterni, Elisa; Donati, Michele; Ciofalo, Andrea; Granata, Guido; Ranuzzi, Patricia; Falasca, Vincenzo; Calvieri, Stefano

doi:10.3390/ijms18122532

Cited by 98 publications

(195 citation statements)

References 96 publications

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“…1 PNP patients show IgG autoantibodies directed against multiple antigens: Dsg3 and/or Dsg1; Dsc1, Dsc2, and Dsc3; proteins of the plakin family (envoplakin, periplakin, desmoplakin I, desmoplakin II, epiplakin, plectin, and BP230), in addition to a protease inhibitor, A2ML1. 1,[4][5][6]15,16,19,20 The reported autoantibody profile is heterogeneous: one study found that antibodies against Dsg3 and Dsg1 are present in 100% and 64% of PNP patients, respectively; the role of these antibodies has been demonstrated and is linked to the induction of blister formation. 21 Another report utilizing ELISA with 79 PNP patients revealed IgG anti-Dsc1, anti-Dsc2, and anti-Dsc3 reactivity of 16.5%, 36.7%, and 59.5%, respectively; the role of these antibodies is still unknown.…”

Section: Antibody-mediated Immunitymentioning

confidence: 99%

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Paraneoplastic pemphigus: a clinical, laboratorial, and therapeutic overview

Maruta

Miyamoto

Aoki

et al. 2019

An. Bras. Dermatol.

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Paraneoplastic 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 α-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.

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Section: Antibody-mediated Immunitymentioning

confidence: 99%

“…Vesicles and blisters are seldom observed. 20 Oral lesions may be the unique expression of PNP. -Cutaneous lesions are characterized as follows: 10,15,34 Erythema multiforme-like lesions: -Graft-versus-host disease: generalized scaly red-brown papules.…”

Section: Oral Lesionsmentioning

confidence: 99%

Paraneoplastic pemphigus: a clinical, laboratorial, and therapeutic overview

Maruta

Miyamoto

Aoki

et al. 2019

An. Bras. Dermatol.

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“…Bone marrow biopsy did not PNP lesions can be detected not only on skin, but also in different mucosal area [2]. Especially, oral mucosa is almost always involved and usually the vermillion border of the lips is included, which resembles the oral lesion of EM [10].…”

Section: Case Reportmentioning

confidence: 91%

Oral Manifestation of Paraneoplastic Pemphigus

Kim

Park

et al. 2019

JOMP

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Paraneoplastic pemphigus (PNP) is a rare and often fatal autoimmune blistering disease accompanied by both benign and malignant neoplasms. Usually, oral, skin, and mucosal lesions are the earliest manifestations shown by PNP patients. Oral ulcers are initial lesions in various autoimmune diseases like pemphigus, bullous pemphigoid, erythema multiforme, graft-versus-host, lichen planus, it does not improved despite of high-dose steroid therapy. We report a-35-year-old female who presented oral ulceration, lip crust and skin lesions. By doing several examinations, such as enzyme-linked immunosorbent assay, incisional biopsy with indirect immunofluorescence, she was diagnosed PNP with non-Hodgkin's lymphoma on pancreas.

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“…There is an erythema multiforme‐like rash on the palms, soles, and extremities, which may show progression to toxic epidermal necrolysis (Figure b). Involvement of the respiratory epithelium is frequently associated with pulmonary disease in the form of bronchiolitis obliterans, a frequently lethal obstructive respiratory disorder .…”

Section: Paraneoplastic Pemphigusmentioning

confidence: 99%

Bullous autoimmune dermatoses

Hofmann

Juratli

Eming

2018

J Deutsche Derma Gesell

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SummaryPathophysiologically, bullous autoimmune dermatoses are caused by autoantibodies directed against adhesion molecules or structural proteins of the skin and mucous membranes, clinically resulting in blister formation. Depending on the respective target proteins of the autoimmune response and their location in the skin, a distinction is made between intraepidermal (pemphigus disorders), junctional (pemphigoid disorders), and subepidermal (epidermolysis bullosa acquisita, dermatitis herpetiformis) autoimmune blistering diseases.The most common bullous autoimmune dermatosis, bullous pemphigoid is characterized by marked clinical variability and intense pruritus. Predominantly affecting elderly individuals, there has been a significant increase in its incidence in recent years. While mucosal lesions occur in less than 30 % of bullous pemphigoid patients, the second most common bullous autoimmune dermatosis, pemphigus vulgaris, typically presents with oral erosions as the predominant and – frequently – initial symptom. Its onset is usually in the 4th to 6th decade of life. Scarring is typically found in subepidermal blistering disorders such as epidermolysis bullosa acquisita or mucous membrane pemphigoid.Diagnosis is based on clinical and histological findings as well as direct and indirect immunofluorescence and detection of circulating autoantibodies. Although a number of controlled clinical trials have been conducted in recent years, treatment of bullous autoimmune disorders is still primarily based on clinical experience. Therapeutic options include topical and systemic corticosteroids as well as adjuvant immunosuppressants. Recalcitrant cases may require treatment with immunoadsorption, intravenous immunoglobulins, or the monoclonal anti‐CD20 antibody rituximab.

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Paraneoplastic Pemphigus: Insight into the Autoimmune Pathogenesis, Clinical Features and Therapy

Cited by 98 publications

References 96 publications

Paraneoplastic pemphigus: a clinical, laboratorial, and therapeutic overview

Paraneoplastic pemphigus: a clinical, laboratorial, and therapeutic overview

Oral Manifestation of Paraneoplastic Pemphigus

Bullous autoimmune dermatoses

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