Neonatal pemphigus vulgaris with extensive mucocutaneous lesions from a mother with oral pemphigus vulgaris

Campo‐Voegeli, A.; Muniz, F. J.; Mascaró, José M.; Casals, Martí; García, F; Arimany, Josep; Amagai, Masayuki; Camps, Anna

doi:10.1046/j.1365-2133.2002.04969.x

Cited by 48 publications

(45 citation statements)

References 41 publications

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“…Leider [31,9]. Der neonatale PV wird durch diaplazentare Übertragung von Autoantikör-pern gegen Desmoglein 3 (Dsg3), hauptsächlich der IgG-4-Klasse ausgelöst [37].…”

Section: Neonataler Pemphigus Foliaceusunclassified

“…Der neonatale PV wird durch diaplazentare Übertragung von Autoantikör-pern gegen Desmoglein 3 (Dsg3), hauptsächlich der IgG-4-Klasse ausgelöst [37]. Dsg3 ist in neonataler Haut stärker als in adulter exprimiert, sodass Hautverände-rungen bei neonatalem PV auch dann auftreten können, wenn eine Mutter mit Schleimhautpemphigus ausschließlich Anti-Dsg3-Antikörper aufweist [50,9]. Allerdings sind bei Müttern von Neugeborenen mit neonatalem PV häufig auch Autoantikörper gegen Dsg1 zu finden, die bei Erwachsenen mit PV zur Auslösung von Blasen an der Haut notwendig sind [37,17].…”

Section: Neonataler Pemphigus Foliaceusunclassified

“…In den wenigen Fällen von Pemphigus vulgaris bei Kindern und Jugendlichen mit ausschließlichem Mundschleimhautbefall können im Serum lediglich Antikörper gegen das klassische PV-Antigen Dsg3 nachweisbar sein, bei Kindern und Jugendlichen mit Haut-und Schleimhautbefall finden sich in der Regel sowohl Antikörper gegen Dsg3 wie Dsg1[9]. Auch die Therapie des juvenilen PV entspricht der im Erwachsenenalter: Am häufigstenAbb.…”

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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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“…Leider [31,9]. Der neonatale PV wird durch diaplazentare Übertragung von Autoantikör-pern gegen Desmoglein 3 (Dsg3), hauptsächlich der IgG-4-Klasse ausgelöst [37].…”

Section: Neonataler Pemphigus Foliaceusunclassified

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Pemphiguserkrankungen bei Kindern und Jugendlichen

Meurer

2009

Hautarzt

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“…Pemphigus foliaceus yes [9][10][11][12][13] yes [14,15] yes [16,17] yes [18] yes [19] IvIg [20] BP180/ collagen XVII…”

Section: Evidence For Pathogenicitymentioning

confidence: 99%

The Role of Pathogenic Autoantibodies in Autoimmunity

Rowley

Whittingham

2015

Antibodies

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Abstract:The serological presence of autoantibodies is diagnostic of autoimmunity, and these autoantibodies may be present for many years before the presentation of autoimmune disease (AID). Although a pathogenic role has been demonstrated for various autoantibodies reactive with cell surface and extracellular autoantigens, studies using monoclonal antibodies (mAb) show not all antibodies in the polyclonal response are pathogenic. Differences depend on Fab-mediated diversity in epitope specificity, Fc-mediated effects based on immunoglobulin (Ig) class and subclass, activation of complement, and the milieu in which the reaction occurs. These autoantibodies often occur in organ-specific AID and this review illustrates their pathogenic and highly specific effects. The role of autoantibodies associated with intracellular antigens is less clear. In vitro they may inhibit or adversely affect well-defined intracellular biochemical pathways, yet, in vivo they are separated from their autoantigens by multiple cellular barriers. Recent evidence that Ig can traverse cell membranes, interact with intracellular proteins, and induce apoptosis has provided new evidence for a pathogenic role for such autoantibodies. An understanding of how autoantibodies behave in the polyclonal response and their role in pathogenesis of AID may help identify populations of culprit B-cells and selection of treatments that suppress or eliminate them.

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“…It initially manifests itself in the oral cavity in 50% of cases [11][12][13] in the form of irregular erosions or ulcers, lesions that are secondary to the breaking of the blisters due to the thinness and friability of their covering. 14,15 Mucosal lesions may be the sole sign for an average of five months before skin lesions develop, or they may be the sole manifestation of the disease.…”

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confidence: 99%