Autoreactivity to BP180 Neoepitopes in Patients With Pemphigoid Gestationis

Schauer, Franziska; Mai, Shoko; Hofmann, Silke; Mai, Yosuke; Izumi, Kiyotaka; Kern, Johannes S.; Kiritsi, Dimitra

doi:10.1001/jamadermatol.2021.5294

Cited by 8 publications

(7 citation statements)

References 8 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…It is a rare AIBD, associated with HLA DR3 and DR4 alleles, and occurs during the 2nd–3rd trimester [ 9 ]. PG is clinically similar to BP and is caused by IgG autoantibodies against BP180 epitopes, but patients with predominantly acral vesicles seem to not present NC16A BP180 antibodies [ 9 , 10 ]. It can be complicated with spontaneous abortions, stillbirth, preterm delivery, and small gestational age, and neonatal PG is seen in 2.8–15% of newborns due to placental transfer of autoantibodies [ 9 ].…”

Section: Discussionmentioning

confidence: 99%

Pseudo-Pemphigoid Gestationis Eruption Following SARS-CoV-2 Vaccination with mRNA Vaccine

2022

View full text Add to dashboard Cite

show abstract

Section: Discussionmentioning

confidence: 99%

Pseudo-Pemphigoid Gestationis Eruption Following SARS-CoV-2 Vaccination with mRNA Vaccine

2022

View full text Add to dashboard Cite

show abstract

“… 142 The disease normally has a self‐limited aspect but reoccurrence is common during additional pregnancies. In PG, IgG (and rarely IgE) autoantibodies are most frequently directed against the main BP antigen BP180 NC16A but also BP230 and other BP180 immunodomains seem to be involved in PG 143 . The diagnosis relies on the same techniques described for BP.…”

Section: Pemphigoid Gestationismentioning

confidence: 99%

“…In PG, IgG (and rarely IgE) autoantibodies are most frequently directed against the main BP antigen BP180 NC16A but also BP230 and other BP180 immunodomains seem to be involved in PG. 143 The diagnosis relies on the same techniques described for BP. A peculiar characteristic of PG is that DIF is mostly C3 + rather than IgG + , due to the prevalent presence of IgG 1 and IgG 3 antibodies which have strong complement-activation properties.…”

Section: Pemphigoid Gestationismentioning

confidence: 99%

Pemphigus and pemphigoids: Clinical presentation, diagnosis and therapy

Didona,

Schmidt,

Maglie

et al. 2023

J Deutsche Derma Gesell

View full text Add to dashboard Cite

SummaryPemphigus and pemphigoid are two potentially life‐threatening groups of autoimmune diseases, characterized by autoantibodies targeting structural components of desmosomes or hemidesmosomes, respectively. Affected patients typically show itchy/painful plaques or blistering skin lesions and/or impairing mucosal blistering and erosions, which may strongly impact their quality of life. Since the milestone work of Walter Lever in 1953, who differentiated these two groups of diseases by histopathological analysis of the level of antibody‐mediated skin cleavage, enormous progresses occurred. Achievements made in laboratory diagnostics now allow to identify antigen specific structural proteins of the skin that are targeted by pathogenic autoantibodies. These progresses were accompanied by an increased understanding of the pathogenesis of these diseases thanks to the establishment of animal models reproducing disease and on studies on skin and blood of affected individuals, which have been leading to novel and disease‐specific treatments. Yet, given their phenotypical overlap with more common dermatological diseases, correct diagnosis and appropriate treatment are often delayed, in some cases leading to irreversible sequelae, including organ dysfunction (i.e., loss of vision in mucous membrane pemphigoid). Here, we provide a concise overview of the clinical appearance, diagnosis and therapeutic management of pemphigus and pemphigoid diseases.

show abstract

“… 142 Die Erkrankung ist normalerweise selbstlimitierend, tritt aber bei Folgeschwangerschaften häufig erneut auf. Beim PG sind IgG‐ (und selten IgE‐)Autoantikörper am häufigsten gegen das BP‐Hauptantigen BP180 NC16A gerichtet, aber auch BP230 und andere BP180‐Immunodomänen scheinen an der Pathogenese des PG beteiligt zu sein 143 . Die Diagnose beruht auf denselben Techniken, die für das BP beschrieben wurden.…”

Section: Pemphigoid Gestationisunclassified

Pemphigus‐ und Pemphigoid‐Erkrankungen: Klinik, Diagnostik und Therapie

Didona,

Schmidt,

Maglie

et al. 2023

J Deutsche Derma Gesell

View full text Add to dashboard Cite

ZusammenfassungPemphigus und Pemphigoid sind seltene Autoimmunkrankheiten der Haut mit potenziell lebensbedrohlichem Verlauf. Autoantikörper gegen epidermale und junktionale Strukturproteine (Desmosomen sowie Hemidesmosomen) führen bei Betroffenen typischerweise zu juckenden, schmerzhaften Plaques oder Blasen an der Haut und/oder Blasenbildung und Erosionen der Schleimhäute mit möglicher Einschränkung der Lebensqualität. Seit der bahnbrechenden Arbeit von Walter Lever im Jahr 1953, dem es gelang, mittels histopathologischer Untersuchung diese beiden Krankheitsgruppen anhand des Musters der Antikörper‐vermittelten Blasenbildung zu differenzieren, wurden enorme Fortschritte im Verständnis der Erkrankungen erzielt. Die Errungenschaften in der Labordiagnostik ermöglichten die Identifikation von Zielstrukturen zur präzisen Unterscheidung verschiedener Varianten der bullösen Autoimmunerkrankungen. Diese Fortschritte gingen dank der Entwicklung von Tiermodellen mit einem besseren Verständnis der Pathogenese einher. Außerdem haben Studien an Haut und Blut betroffener Patienten zu neuen und krankheitsspezifischen Behandlungen geführt. Aufgrund ihrer Seltenheit und der klinischen Ähnlichkeit mit anderen dermatologischen Erkrankungen verzögern sich die korrekte Diagnosestellung und die Einleitung einer entsprechenden Therapie häufig, was in einigen Fällen zu irreversiblen Folgeerscheinungen, einschließlich Funktionsstörungen von Organen (zum Beispiel Verlust des Sehvermögens beim Schleimhautpemphigoid) führt. Wir geben hier einen Überblick über das klinische Erscheinungsbild, den Diagnosealgorithmus und das therapeutische Management von Pemphigus‐ und Pemphigoid‐Erkrankungen.

show abstract

Autoreactivity to BP180 Neoepitopes in Patients With Pemphigoid Gestationis

Cited by 8 publications

References 8 publications

Pseudo-Pemphigoid Gestationis Eruption Following SARS-CoV-2 Vaccination with mRNA Vaccine

Pseudo-Pemphigoid Gestationis Eruption Following SARS-CoV-2 Vaccination with mRNA Vaccine

Pemphigus and pemphigoids: Clinical presentation, diagnosis and therapy

Pemphigus‐ und Pemphigoid‐Erkrankungen: Klinik, Diagnostik und Therapie

Contact Info

Product

Resources

About