Neonatal pemphigus vulgaris: IgG4 autoantibodies to desmoglein 3 induce skin blisters in newborns

Parlowsky, Thomas; Welzel, Julia; Amagai, Masayuki; Zillikens, Detlef; Wygold, T.

doi:10.1067/mjd.2003.170

Cited by 53 publications

(36 citation statements)

References 9 publications

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“…There is a potential comorbidity with bipolar affective disorder and other neuropsychiatric illness. 2 There are at least 9 previous reports of cystic bone disease. [3][4][5][6][7][8][9][10][11] We describe an additional case of bone cysts in an adolescent boy with severe skin involvement.…”

Section: Cystic Bone Lesions In a Boy With Darier Diseasementioning

confidence: 98%

“…1 Neonatal pemphigus is a rarely reported transitory autoimmune blistering disease caused by transfer of maternal IgG autoantibodies to desmoglein 3 to the neonate through the placenta when the mother is affected with pemphigus. 2 It is clinically characterized by transient flaccid blisters and erosions on the skin and rarely the mucous membranes. 3 Neonatal pemphigus vulgaris has never been reported to persist beyond the neonatal period and progress to adult disease.…”

Section: Introductionmentioning

confidence: 99%

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Neonatal pemphigus in an infant born to a mother with serologic evidence of both pemphigus vulgaris and gestational pemphigoid

Panko

Florell

Hadley

et al. 2009

Journal of the American Academy of Dermatology

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Section: Cystic Bone Lesions In a Boy With Darier Diseasementioning

confidence: 98%

Section: Introductionmentioning

confidence: 99%

Neonatal pemphigus in an infant born to a mother with serologic evidence of both pemphigus vulgaris and gestational pemphigoid

Panko

Florell

Hadley

et al. 2009

Journal of the American Academy of Dermatology

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“…Neonatal pemphigus is thought to be caused by the transfer of maternal IgG autoantibodies to desmoglein 3 and is characterized by transient severe blisters and erosions in the skin [20]. Clinical manifestations of neonatal myasthenia gravis include muscle weakness, poor suckling, respiratory disease, and facial diplegia.…”

Section: Maternal Antibodies and Developmental Abnormalities: Human Smentioning

confidence: 99%

Maternal antibodies and developing blood–brain barrier

et al. 2015

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We briefly review the protective role of maternal antibodies during fetal development and at early postnatal stages. We describe antibody delivery to fetuses, particularly in the context of the developing blood–brain barrier (BBB), and present the essential concepts regarding the adult BBB, together with existing information on the prenatal developing BBB. We focus on maternal antibody transfer to the developing brain and the consequences of the presence of pathogenic antibodies at early stages of brain development on subsequent brain dysfunction.

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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]. 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].…”

Section: Neonataler Pemphigus Foliaceusunclassified

“…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

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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Neonatal pemphigus vulgaris: IgG4 autoantibodies to desmoglein 3 induce skin blisters in newborns

Cited by 53 publications

References 9 publications

Neonatal pemphigus in an infant born to a mother with serologic evidence of both pemphigus vulgaris and gestational pemphigoid

Neonatal pemphigus in an infant born to a mother with serologic evidence of both pemphigus vulgaris and gestational pemphigoid

Maternal antibodies and developing blood–brain barrier

Pemphiguserkrankungen bei Kindern und Jugendlichen

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