[Pemphigoid diseases in older adults].
Pemphigoiderkrankungen bei älteren Menschen.
Anti-p200 pemphigoid
Autoantibodies
Bullous pemphigoid
Immunofluorescence
Mucous membrane pemphigoid
Journal
Dermatologie (Heidelberg, Germany)
ISSN: 2731-7013
Titre abrégé: Dermatologie (Heidelb)
Pays: Germany
ID NLM: 9918384885206676
Informations de publication
Date de publication:
Sep 2023
Sep 2023
Historique:
accepted:
18
07
2023
medline:
6
9
2023
pubmed:
18
8
2023
entrez:
18
8
2023
Statut:
ppublish
Résumé
Pemphigoid diseases are a group of bullous autoimmune diseases characterized by autoantibodies against structural proteins of the dermal-epidermal junction. With a steadily growing aging population, pemphigoid diseases are emerging as a significant medical challenge, because they occur primarily in older individuals. The by far most common disease is bullous pemphigoid, which is clinically characterized by tense blisters, erosions, erythema or urticarial plaques, while severe pruritus is the leading subjective symptom. Mucous membrane pemphigoid predominantly affects surface-close mucous membranes with painful erosions and blisters as well as frequently scarring usually in the mouth, nose, and eyes. Anti-p200 pemphigoid clinically resembles bullous pemphigoid but is much less common. Diagnosis of these diseases involves the combination of clinical evaluation, lesional histopathology, direct immunofluorescence microscopy of a perilesional biopsy and serology. Topical and systemic corticosteroids are the mainstay of pemphigoid diseases treatment. Depending on the severity of the disease, various potentially corticosteroid-sparing therapies, such as dapsone, doxycycline, methotrexate, azathioprine and mycophenolate may be used. In severe courses, treatment with rituximab, cyclophosphamide, intravenous immunoglobulins or immunoadsorption are second- or third-line treatment options. Patients are best managed in centers experience with the management of pemphigoid diseases. Updated national and international guidelines for the diagnosis and treatment of bullous pemphigoid and mucous membrane pemphigoid have recently been published. Pemphigoiderkrankungen sind eine Gruppe von blasenbildenden Autoimmundermatosen mit Bildung von Autoantikörpern gegen Strukturproteine der dermoepidermalen Junktionszone. Mit einer zunehmend alternden Bevölkerung gewinnen die Pemphigoiderkrankungen an Bedeutung, da sie v. a. bei älteren Personen auftreten. Die mit Abstand häufigste Erkrankung ist das bullöse Pemphigoid, das sich klinisch durch pralle Blasen, Erosionen, Erytheme, urtikarielle Plaques sowie starken Pruritus auszeichnet. Beim Schleimhautpemphigoid kommt es an den oberflächennahen Schleimhäuten, meist von Mund, Nase und Augen, zu schmerzhaften Erosionen und Blasen sowie Vernarbungen. Das Anti-p200-Pemphigoid ähnelt klinisch dem bullösen Pemphigoid, ist jedoch deutlich seltener. Für die Diagnosestellung ist neben der Klinik eine entsprechende Diagnostik mit Histopathologie, direkter Immunfluoreszenz einer periläsionalen Biopsie und Serologie entscheidend. Je nach Schweregrad der Erkrankung werden neben topischen und systemischen Kortikosteroiden verschiedene potenziell Kortikosteroid-sparende Therapien eingesetzt. Hierzu zählen v. a. Dapson, Doxycyclin, Methotrexat, Azathioprin und Mycophenolat. Bei schweren Verläufen kann eine Therapie mit Rituximab, Cyclophosphamid, intravenösen Immunglobulinen oder Immunadsorption in Erwägung gezogen werden. Die Behandlung sollte in entsprechend spezialisierten Zentren durchgeführt werden. Für das bullöse Pemphigoid und das Schleimhautpemphigoid stehen mittlerweile nationale (AWMF [Arbeitsgemeinschaft der Wissenschaftlichen Medizinischen Fachgesellschaften e. V.]) und internationale Leitlinien zur Verfügung.
Autres résumés
Type: Publisher
(ger)
Pemphigoiderkrankungen sind eine Gruppe von blasenbildenden Autoimmundermatosen mit Bildung von Autoantikörpern gegen Strukturproteine der dermoepidermalen Junktionszone. Mit einer zunehmend alternden Bevölkerung gewinnen die Pemphigoiderkrankungen an Bedeutung, da sie v. a. bei älteren Personen auftreten. Die mit Abstand häufigste Erkrankung ist das bullöse Pemphigoid, das sich klinisch durch pralle Blasen, Erosionen, Erytheme, urtikarielle Plaques sowie starken Pruritus auszeichnet. Beim Schleimhautpemphigoid kommt es an den oberflächennahen Schleimhäuten, meist von Mund, Nase und Augen, zu schmerzhaften Erosionen und Blasen sowie Vernarbungen. Das Anti-p200-Pemphigoid ähnelt klinisch dem bullösen Pemphigoid, ist jedoch deutlich seltener. Für die Diagnosestellung ist neben der Klinik eine entsprechende Diagnostik mit Histopathologie, direkter Immunfluoreszenz einer periläsionalen Biopsie und Serologie entscheidend. Je nach Schweregrad der Erkrankung werden neben topischen und systemischen Kortikosteroiden verschiedene potenziell Kortikosteroid-sparende Therapien eingesetzt. Hierzu zählen v. a. Dapson, Doxycyclin, Methotrexat, Azathioprin und Mycophenolat. Bei schweren Verläufen kann eine Therapie mit Rituximab, Cyclophosphamid, intravenösen Immunglobulinen oder Immunadsorption in Erwägung gezogen werden. Die Behandlung sollte in entsprechend spezialisierten Zentren durchgeführt werden. Für das bullöse Pemphigoid und das Schleimhautpemphigoid stehen mittlerweile nationale (AWMF [Arbeitsgemeinschaft der Wissenschaftlichen Medizinischen Fachgesellschaften e. V.]) und internationale Leitlinien zur Verfügung.
Identifiants
pubmed: 37594515
doi: 10.1007/s00105-023-05209-3
pii: 10.1007/s00105-023-05209-3
doi:
Substances chimiques
Immunosuppressive Agents
0
Types de publication
English Abstract
Journal Article
Review
Langues
ger
Sous-ensembles de citation
IM
Pagination
687-695Informations de copyright
© 2023. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.
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