Atypical Clinical and Serological Manifestation of Pemphigus Vegetans: A Case Report and Review of the Literature

Mergler, Rebecca; Kerstan, Andreas; Schmidt, Enno; Goebeler, Matthias; Benoit, Sandrine

doi:10.1159/000468919

Cited by 29 publications

(27 citation statements)

References 37 publications

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“…Treatment is the same as for the more common pemphigus variants. Given the specific sites involved, intensive topical treatment with antiseptics is required .…”

Section: Pemphigus Vegetansmentioning

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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“…Treatment is the same as for the more common pemphigus variants. Given the specific sites involved, intensive topical treatment with antiseptics is required .…”

Section: Pemphigus Vegetansmentioning

confidence: 99%

Bullous autoimmune dermatoses

Hofmann

Juratli

Eming

2018

J Deutsche Derma Gesell

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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%

“…As P veg is a subtype of PV, it is expected to react with the same antigens, Dsg1 and Dsg3 [2,5,50]. The presence of auto-Abs targeting additional desmosomal proteins including Dsc1, Dsc2, Dsc3 and periplakin have also been reported [51,55].…”

Section: Immunopathology and Target Antigensmentioning

confidence: 99%

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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“…Вегетирующая пузырчатка -аутоиммунное буллезное заболевание кожи, развивающееся в результате продукции аутоантител к белкам десмосомального аппарата клеток эпидермиса и слизистых оболочек, вызывающих развитие акантолиза, характеризующееся формированием вегетаций на образующихся после вскрытия пузырей эрозиях [1]. Согласно международной классификации болезней ВОЗ 10-го пересмотра, вегетирующая пузырчатка является вариантом пемфигуса.…”

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“…R. Mergler и соавт. (2017) указывают, что не всегда клинические проявления вегетирующей пузырчатки позволяют установить тип заболевания [1].…”

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