Brasilianischer Pemphigus foliaceus (Fogo selvagem)

Kunte, Christian; Barbosa, Josiane M.; Wolff, Hans; Meurer, Michael

doi:10.1007/s001050050574

Cited by 9 publications

(7 citation statements)

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“…The oral mucosa, palms of the hands, and plant of the feet are usually spared [59,66]. In both PF and FS, lesions may become confluent and can transform to exfoliative erythroderma [67]. These patients should be hospitalized due to the risk of metabolic instability and mortality [1,16,59].…”

Section: Clinical Featuresmentioning

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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Section: Clinical Featuresmentioning

confidence: 99%

Pemphigus: Subtypes, Clinical Features, Diagnosis, and Treatment

Kılıç¹

2018

Autoimmune Bullous Diseases

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“…Fast immer sind diese Ansiedlungen an Flüssen gelegen. Typische Beispiele hierfür sind der Parana und Paranapanema im Süden sowie der Tocantins und Araguaia im Norden: In anderen süd-amerikanischen Staaten wie Argentinien, Bolivien, Paraguay, Peru und Kolumbien werden Fälle von endemischem PF nur selten beobachtet, hier kann die Unterscheidung von dem bei uns vorkommenden nichtendemischen PF schwierig sein [22].…”

Section: Endemischer Pemphigus Foliaceus Bei Kindern Und Jugendlichenunclassified

“…B. mit Immunsuppressiva oder Dapson. In den südamerikanischen Endemiegebieten ist bei den jugendlichen steroidbehandelten Patienten auf die Entwicklung oder Exazerbation einer Tuberkulose besonders zu achten [22,38].…”

Section: Therapie Des Endemischen Pemphigus Foliaceusunclassified

Pemphiguserkrankungen bei Kindern und Jugendlichen

Meurer

2009

Hautarzt

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The pemphigus diseases, which include some of the most severe bullous autoimmune skin reactions, are seen predominantly in middle-aged and elderly individuals. Only endemic pemphigus foliaceus in South America most frequently affects juveniles and children. All non-endemic pemphigus diseases, including paraneoplastic pemphigus, have been reported to occur in adolescents and even very rarely in children younger than 10 years. Pemphigus vulgaris in pregnancy represents a frequently overseen medical problem and may result in fetal growth retardation, intrauterine death, premature delivery and - in about 30% - in neonatal pemphigus vulgaris of the newborn. Contrary to pemphigus vulgaris, the transplacental crossing of autoantibodies against desmoglein1 in pregnant women with pemphigus foliaceus hardly ever leads to neonatal skin lesions in the offspring. This phenomenon can be explained by differences in the distribution and cross-compensation of the pemphigus antigens desmoglein3 and 1 in neonatal and adult skin or mucosa, respectively.

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“…Dieses Phänomen wurde kürzlich mit Hilfe der Dsg-3-Knock-out-Maus (Dsg 3-/-) untersucht, deren Keratinozyten kein Dsg 3 exprimieren. Die südamerikanische Form des endemischen Pemphigus wird auch als brasilianischer Pemphigus oder Fogo selvagem ("wildes Feuer") bezeichnet, unterscheidet sich aber klinisch, histologisch und immunpathologisch nicht von der idiopathischen Form des PF [21,47] [11]. Werden geringe Mengen von Antikörpern gegen Dsg 1 in gesunde Dsg 3+/+ Mäuse injiziert, kommt es zu keiner Blasenbildung an der Haut.…”

Section: Bei Pv Und Pf Hängt Die Beteiligung Von Haut Oder Schleimhauunclassified

Pemphigus

Schmidt¹,

Eb²,

Zillikens³

2000

Der Hautarzt

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Pemphigus diseases comprise a group of autoimmune disorders which are characterized by intraepidermal blisters and autoantibodies to components of desmosomes. Desmosomes mediate adhesion between neighbouring keratinocytes. A common feature of pemphigus diseases are intercellular deposits of IgG or, less frequently, of IgA within the epidermis. The group of pemphigus diseases includes pemphigus vulgaris, pemphigus foliaceus, pemphigus vegetans, pemphigus herpetiformis, pemphigus erythematosus, paraneoplastic pemphigus, drug-induced pemphigus, and IgA pemphigus. Using molecular tools, some of the autoantigens in these diseases have been characterized. In pemphigus vulgaris, autoantibodies are directed to desmoglein 3 and in pemphigus foliaceus to desmoglein 1. Target antigens in IgA pemphigus are desmocollin 1 and desmoglein 3. In paraneoplastic pemphigus, autoantibodies react with a complex of various proteins, including desmoplakin 1 and 2, BP230, envoplakin, periplakin, plectin, desmoglein 3, and a yet uncharacterized 170 kD protein. This review summarizes new insights into the immunopathogenesis and diagnosis of pemphigus diseases.

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Brasilianischer Pemphigus foliaceus (Fogo selvagem)

Cited by 9 publications

References 38 publications

Pemphigus: Subtypes, Clinical Features, Diagnosis, and Treatment

Pemphigus: Subtypes, Clinical Features, Diagnosis, and Treatment

Pemphiguserkrankungen bei Kindern und Jugendlichen

Pemphigus

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