Pemphigus vegetans of the folds (intertriginous areas)

Ruocco, Vincenzo; Ruocco, Eleonora; Caccavale, Stefano; Gambardella, Alessio; Schiavo, Ada Lo

doi:10.1016/j.clindermatol.2015.04.011

Cited by 46 publications

(71 citation statements)

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“…P veg is characterized by vegetative lesions preferentially affecting intertriginous (axillary, inframammarial areas) and periorificial regions [2,5,[50][51][52]. The initial course of the disease is similar to PV.…”

Section: Clinical Featuresmentioning

confidence: 99%

“…Involvement of the vermillion border of the lips is the clinical hallmark of oral involvement [54]. Nail involvement is rarely described [50]. In P veg, the course of the disease is long, with remission and recurrence periods.…”

Section: Clinical Featuresmentioning

confidence: 99%

“…P veg has been reported to occur in all age groups, affecting primarily middle-aged females (sex ratio: F/M = 14/3) [50]. Autoimmune Bullous Diseases…”

Section: Pemphigus Vegetansmentioning

confidence: 99%

“…The initial course of the disease is similar to PV. In the later stages, tumid vegetating, hypertrophic and verrucous lesions occur specifically between skin folds [5,50,53]. Two subtypes of P veg are recognized.…”

Section: Clinical Featuresmentioning

confidence: 99%

“…The first one is Neumann P veg, which usually begins like PV with easily rupturing vesicles and bullae that evolve to form hypertrophic granulating erosions and then vegetating exuding masses. The second type is Hallopeau P veg, which is initially characterized by pustular lesions that break and gradually evolve into vegetating erosions [5,50]. Mucosal involvement may not always be seen.…”

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: Clinical Featuresmentioning

confidence: 99%

Section: Clinical Featuresmentioning

confidence: 99%

“…P veg has been reported to occur in all age groups, affecting primarily middle-aged females (sex ratio: F/M = 14/3) [50]. Autoimmune Bullous Diseases…”

Section: Pemphigus Vegetansmentioning

confidence: 99%

Section: Clinical Featuresmentioning

confidence: 99%

Section: Clinical Featuresmentioning

confidence: 99%

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