Paraneoplastic autoimmune multiorgan syndrome: 20 years after

Czernik, Annette; Camilleri, Michael; Pittelkow, Mark R.; Grando, Sergei A.

doi:10.1111/j.1365-4632.2011.04868.x

Cited by 97 publications

(144 citation statements)

References 68 publications

(145 reference statements)

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“…The histopathological features of PNP reveal variability according to the type of the morphology of the cutaneous lesion [15,77,84,90]. Intraepidermal suprabasal acantholysis (resembling PV), keratinocyte necrosis and vacuolar interface changes (resembling erythema multiforme/lichen planus) may be observed [77,90].…”

Section: Histopathologymentioning

confidence: 99%

“…As though most of the patients with PNP are associated with malignancies, the prognosis of PNP is severe with a high mortality rate [15,77,83,84,88,89]. Internal organ involvement including lungs (Bronchiolitis obliterans), thyroid, kidney and gastrointestinal system has been documented [15,88,89].…”

Section: Clinical Featuresmentioning

confidence: 99%

“…Eye involvement especially includes conjunctival erosions and occurs in 70% of patients [79][80][81][82]. Cutaneous lesions are usually seen after the onset of mucosal involvement with a duration of days to months [77,83,84]. Cutaneous lesions are widespread and are usually polymorphic including lichenoid lesions, erythema multiforme-like lesions, vesiculobullous and erosive lesions.…”

Section: Clinical Featuresmentioning

confidence: 99%

“…The palmar involvement is usually observed [4]. Lichen planus-like lesions localized on skin, nails and/or mucosa resemble lichen planus, target-like lesions resemble erythema multiforme, and bullous lesions and erosive lesions resemble PV and bullous pemphigoid [4,15,66,76,77,[83][84][85][86][87]. Cutaneous lesions mimicking graft versus host disease or Stevens-Johnson syndrome may also be observed [86,87].…”

Section: Clinical Featuresmentioning

confidence: 99%

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Pemphigus: Subtypes, Clinical Features, Diagnosis, and Treatment

Kılıç¹

2018

Autoimmune Bullous Diseases

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Pemphigus is a group of autoimmune blistering disorders associated with autoantibodies against the keratinocyte cell surface. Pemphigus has three major variants: pemphigus vulgaris (PV), pemphigus foliaceus (PF), and paraneoplastic pemphigus (PNP) which all have further subtypes. The variants of pemphigus are classified depending upon the clinical and histological features, immunofluorescence staining pattern, and autoantibody profile of the disease. The onset and course of pemphigus appear on the basis of interaction between genetic predisposition and various triggering factors. Pemphigus vulgaris is the most commonly seen and representative clinical form of pemphigus. Together with clinical manifestations, the histopathological and immunopathological data support the diagnosis. As though some pemphigus variants, particularly pemphigus vulgaris and paraneoplastic pemphigus, have a mortality risk, early diagnosis is necessary and onset of treatment should be promptly initiated. In this chapter, firstly, classification of pemphigus is described. After then, clinical features, histopathological and immunopathological findings, target antigens, etiopathogenesis and comorbidities of each pemphigus variant are discussed briefly.Keywords: pemphigus, pemphigus variants, clinical manifestations, pathogenesis, histopathology, immunopathology IntroductionPemphigus is a distinct organ-specific autoimmune blistering disorder involving skin and mucous membranes associated with autoantibodies directed against desmosomes-intercellular adhesive molecule complex localized on the keratinocyte cell surface [1][2][3][4].© 2018 The Author(s). Licensee IntechOpen. This chapter is distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.Pemphigus has three major variants, which are classified depending on the basis of the clinical, histological features, immunofluorescence staining pattern and autoantibody profile of the disease including pemphigus vulgaris (PV), pemphigus foliaceus (PF), and paraneoplastic pemphigus (PNP), which all have further subtypes. Less frequently seen and newer variants of pemphigus include IgA pemphigus (IGAP) and pemphigus herpetiformis (PH) [1-6].The term "pemphigus" origins from the Greek word "pemphix", which has a meaning of "blister" [1]. It is a chronic potentially life-threating bullous disorder if not treated on time [4,7,8]. The phenotypes of pemphigus represent a complex spectrum with multiple genetic and environmental factors playing a role in disease pathogenesis [9,10].Together with clinical manifestations, the histopathological and immunopathological data support the diagnosis of the disease. The best site for the cutaneous biopsy for the appropriate histopathological examination is a fresh (< 24 h) small vesicle or 1/3 of the peripheral portion of the blister including the perilesional normal appearing s...

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Section: Histopathologymentioning

confidence: 99%

Section: Clinical Featuresmentioning

confidence: 99%

Section: Clinical Featuresmentioning

confidence: 99%

Section: Clinical Featuresmentioning

confidence: 99%

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Pemphigus: Subtypes, Clinical Features, Diagnosis, and Treatment

Kılıç¹

2018

Autoimmune Bullous Diseases

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“…Indeed, the involvement of the palms and soles is common in pemphigoid diseases 7 . When PNP affects the oral mucosa, the PNP is far more diffuse and severe than that observed in pemphigus vulgaris, resulting in erosive stomatitis 8 .…”

mentioning

confidence: 99%

Paraneoplastic autoimmune multi-organ syndrome is a distinct entity from traditional pemphigus subtypes

Amber

2018

Nat Rev Dis Primers

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Paraneoplastic Autoimmune Multiorgan Syndrome in a Patient With Li Fraumeni Syndrome

O’Gorman

Torgerson

2013

JAMA Dermatol

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PAMS is a heterogeneous condition mediated by either a predominantly autoantibody or cell-mediated cytotoxic (CMC) effect. The predominance of a CMC mechanism is associated with the lichenoid variant. 2,3 The best outcomes are seen in cases where the underlying malignant neoplasm is fully resectable, with resection leading to improvement in 15 of 20 patients in one series. 4 Unfortunately, our patient did not dem-

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Paraneoplastic autoimmune multiorgan syndrome: 20 years after

Cited by 97 publications

References 68 publications

Pemphigus: Subtypes, Clinical Features, Diagnosis, and Treatment

Pemphigus: Subtypes, Clinical Features, Diagnosis, and Treatment

Paraneoplastic autoimmune multi-organ syndrome is a distinct entity from traditional pemphigus subtypes

Paraneoplastic Autoimmune Multiorgan Syndrome in a Patient With Li Fraumeni Syndrome

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