Hallopeau type of pemphigus vegetans, an unusual clinical presentation of an infrequent disease

Cuellar, Isabel; Mejía, Maddy; Castellanos-Angarita, Alejandro; Casas-Barrera, María Alejandra

doi:10.5070/d32610050470

Cited by 7 publications

(4 citation statements)

References 6 publications

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“…It is different from pemphigus vulgaris as patients develop vegetative lesions occurring most frequently in intertriginous spaces [9]. The data in the literature describe two subtypes: the Hallopeau type, a milder variant that usually presents with initial pustules that transform into vegetative, pain-inducing lesions, and the Neumann subtype, a more aggressive presentation characterized by vesicles, bullae, and ulcerative lesions which in time become verrucous [10,11]. The case that we have presented correlates with the Neumann subtype, due to its clinical progress from large erosions to severe vegetating plaques composed of granulation tissue and crusts that covered the entire upper part of the trunk and the neck area.…”

Section: Discussionmentioning

confidence: 99%

“…Numerous investigations have elucidated specific associations between PV/PF and certain human leukocyte antigen (HLA) class II alleles [10]. For instance, studies have revealed a heightened prevalence of PV among the Ashkenazi Jewish population possessing the HLA-DRB1 0402 allele in comparison to other demographic groups [11]. Conversely, Lombardi et al demonstrated that, in Italian patients, PV and PF share similar HLA alleles, suggesting additional factors (environment, drug exposure, dietary, etc.)…”

Section: Introductionmentioning

confidence: 99%

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The Transition from Pemphigus Foliaceus to Pemphigus Vegetans—An Intriguing Phenomenon within the Spectrum of Autoimmune Blistering Diseases: A Case Report

Orzan,

Popa,

Badiu

et al. 2024

Dermato

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Pemphigus vegetans and pemphigus foliaceus are rare autoimmune blistering diseases characterized by the disruption of desmosomal adhesion proteins, particularly desmoglein 3 and desmoglein 1. We report the case of a 62-year-old male who presented initially with scaly red plaques posing several diagnostic challenges. A histopathological examination revealed subcorneal acantholysis, matching the suspected clinical diagnosis of pemphigus foliaceus. The patient progressed, developing vegetating plaques, and a new biopsy was performed. The new histopathological and direct immunofluorescence exams were consistent with pemphigus vegetans. This case highlights the diagnostic challenges posed by the transition of pemphigus foliaceus to its vegetating form. We discuss the role of desmogleins in the pathogenesis of pemphigus and explore potential therapeutic strategies targeting these specific autoantigens.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

The Transition from Pemphigus Foliaceus to Pemphigus Vegetans—An Intriguing Phenomenon within the Spectrum of Autoimmune Blistering Diseases: A Case Report

Orzan,

Popa,

Badiu

et al. 2024

Dermato

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“…Neumann type is characterized by bullae that extend and coalesce, evolving into vegetating masses (3). Hallopeau-type PVeg is characterized by a polycyclic eruption of pustules forming firm pink papilloma that progressively flatten, with a benign course and few relapses (3,4). PVeg usually affects younger adults or middle-aged females (5).…”

Section: Introductionmentioning

confidence: 99%

Pemphigus vegetans Hallopeau with nail changes and no oral involvement

Tomanović,

Lalošević,

Škiljević

et al. 2023

Medicinska istraživanja

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Introduction: Pemphigus vegetans (PVeg) is the rarest form of autoimmune pemphigus. Lesions are primarily flexural and mucosal, although they may occur at any site. Oral involvement is common. Two subtypes are recognized - the Neumann type and the less common Hallopeau type. Patient review: We present a Hallopeau-type PVeg with no oral lesions and with uncommon nail changes. In the follow-up period of ten years, the patient experienced several flares but no changes in the oral cavity. Conclusion: This case is interesting due to the disease's rarity, atypic clinical presentation, and an association with multiple pregnancies as a possible precipitating factor.

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“…Dados epidemiológicos sobre o pênfigo vegetante são escassos, mas o acometimento é maior em mulheres com uma proporção de 1,3 -2,3 para 1 em relação ao sexo masculino. A idade de diagnóstico costuma variar entre 40 e 60 anos (CUELLAR et al, 2020).…”

Section: Introductionunclassified

Pênfigo vegetante de hallopeau em puerpério imediato: relato de caso e breve revisão de literatura/ Hallopeau vegetant pemphigus in immediate puerperia: case report and brief literature review

Bergamin¹,

França²,

Farias³

et al. 2022

Braz. J. Hea. Rev.

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RESUMOO pênfigo de Hallopeau é uma enfermidade do grupo dos pênfigos vegetantes, sendo estes, variantes raras do pênfigo vulgar. Clinicamente, o pênfigo vegetante de Hallopeau manifestase com pústulas que, normalmente, evoluem com a formação de placas vegetantes sem acometimento mucoso. Embora dados epidemiológicos sejam escassos, há certo predomínio deste acometimento na população feminina em relação à masculina. A fisiopatologia da doença é determinada pelos autoanticorpos IgG contra as proteínas transmembrana que formam os desmossomos, os quais são os responsáveis pela adesão célula-célula dos queratinócitos, principalmente contra a desmogleína 3 (DSG3) e a desmogleína 1 (DSG1), o que, consequentemente, leva à acantólise, ou seja, à separação da camada espinhosa da epiderme. Os fatores desencadeantes deste tipo de pênfigo ainda não estão totalmente esclarecidos, mas acredita-se que a gravidez possa ser um deles devido ao rápido aumento nos níveis de estrogênio, seguido por uma alteração no equilíbrio da relação de linfócitos Th1/Th2 com aumento de Th2. Este aumento também leva a maior produção de autoanticorpos IgG, como resultado de um aumento das citocinas relacionadas ao IgG. Em alguns casos com associação

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Hallopeau type of pemphigus vegetans, an unusual clinical presentation of an infrequent disease

Cited by 7 publications

References 6 publications

The Transition from Pemphigus Foliaceus to Pemphigus Vegetans—An Intriguing Phenomenon within the Spectrum of Autoimmune Blistering Diseases: A Case Report

The Transition from Pemphigus Foliaceus to Pemphigus Vegetans—An Intriguing Phenomenon within the Spectrum of Autoimmune Blistering Diseases: A Case Report

Pemphigus vegetans Hallopeau with nail changes and no oral involvement

Pênfigo vegetante de hallopeau em puerpério imediato: relato de caso e breve revisão de literatura/ Hallopeau vegetant pemphigus in immediate puerperia: case report and brief literature review

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