Production of numerous autoantibodies in paraneoplastic pemphigus

Hashimoto, Takashi

doi:10.1111/bjd.13648

Cited by 12 publications

(8 citation statements)

References 13 publications

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“…The mortality rate of PNP is high (90%) [11]. It may also be associated with myasthenia gravis and thymomas [22].…”

Section: Mechanism Of Acantholysismentioning

confidence: 99%

“…In PNP, targets of autoantibodies are more than one: dsgs, dscs, DP1 and 2, bullous pemphigoid antigen (BPAg)1, PF, PP, envoplakin, plectin, epiplakin and alpha-2-macroglobulin-like-1 (A2ML-1) that is a broad range protease inhibitor expressed stratified epithelia and other damaged tissues in PNP [11,20,22]. Characteristic autoantibodies in PNP target the plakin family proteins that are molecules localized in the intracellular plaque of desmosomes and hemidesmosomes [20].…”

Section: Mechanism Of Acantholysismentioning

confidence: 99%

“…High levels of these autoantibodies correlated with dyspnea in PNP patients. These antibodies target not only epidermal proteins but also other antigens in neural and bronchial tissues [22].…”

Section: Mechanism Of Acantholysismentioning

confidence: 99%

“…In patients associated with thymoma, it has been thought that defective thymocyte maturation might lead to the production of autoreactive T cells that can induce B-cell proliferation and autoantibody production. In hematologic malign tumors, aberrant immunological conditions caused by tumors might cause the production of many autoantibodies [22]. Another theory is that autoantibodies against the neoplastic antigens cross-react to epithelial antigens [21].…”

Section: Mechanism Of Acantholysismentioning

confidence: 99%

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Introduction to Autoimmune Bullous Diseases

Gönül¹,

Çakmak²

2018

Autoimmune Bullous Diseases

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Autoimmune bullous diseases are heterogeneous group of disorders characterized by intraepidermal and subepidermal bullae formation. Autoantibodies to major players of skin integrity cause devastating symptoms in autoimmune bullous diseases that may result with morbidity and even mortality in the affected patients. These group of diseases can be categorized by the level of splitting in the skin and by structural proteins that are targeted by autoantibodies. Autoimmune bullous diseases can be divided into four basic subgroups: pemphigus, pemphigoid, epidermolysis bullosa acquisita and dermatitis herpetiformis, although their different subtypes have been defined. In this chapter, the structure and tasks of desmosomes and basement membrane zone, which consist of the major antigens of the skin integrity targeted by autoantibodies, are examined, and the relation of target antigens and autoimmune bullous diseases is discussed.

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“…The mortality rate of PNP is high (90%) [11]. It may also be associated with myasthenia gravis and thymomas [22].…”

Section: Mechanism Of Acantholysismentioning

confidence: 99%

Section: Mechanism Of Acantholysismentioning

confidence: 99%

Section: Mechanism Of Acantholysismentioning

confidence: 99%

Section: Mechanism Of Acantholysismentioning

confidence: 99%

See 2 more Smart Citations

Introduction to Autoimmune Bullous Diseases

Gönül¹,

Çakmak²

2018

Autoimmune Bullous Diseases

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“…ELISA may show autoantibodies to desmoglein 3 and, less often, desmoglein 1. 14 New, commercially available ELISA testing to envoplakin may increase the sensitivity and specificity of serologic testing to surpass that of current immunofluorescence testing. Using immunoprecipitation and mass spectrometry, the 170 kD antigen target has been identified as a protease inhibitor, α-2 macroglobulin-like protein, a broad range protease inhibitor expressed in stratified epithelia and other tissues damaged in PNP.…”

Section: Immunologymentioning

confidence: 99%

Paraneoplastic pemphigus: a short review

Wieczorek¹,

Czernik²

2016

CCID

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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 between 45 and 70 years old, but it also occurs in children and adolescents. A wide variety of lesions (florid oral mucosal lesions, a generalized polymorphous cutaneous eruption, and pulmonary involvement) may occur in patients with PNP. The earliest and most consistent finding is severe stomatitis. There is a spectrum of at least five clinical variants with different morphology. Similarly, the histological findings are very variable. Investigations to diagnose PNP should include checking 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 and respiratory tract. Treatment of PNP is difficult, and the best outcomes have been reported with benign neoplasms that have been surgically excised. The first-line treatment is high-dose corticosteroids with the addition of steroid-sparing agents. Treatment failures are often managed with rituximab with or without concomitant intravenous immunoglobulin. In general, the prognosis is poor, not only because of eventual progression of malignant tumors but also because treatment with aggressive immunosuppression therapy often results in infectious complications, which is unfortunately at this time the most common cause of death in PNP.

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Paraneoplastic Pemphigus

Hashimoto

2021

Diseases of the Oral Mucosa

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Production of numerous autoantibodies in paraneoplastic pemphigus

Cited by 12 publications

References 13 publications

Introduction to Autoimmune Bullous Diseases

Introduction to Autoimmune Bullous Diseases

Paraneoplastic pemphigus: a short review

Paraneoplastic Pemphigus

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