Bullous pemphigoid
Bullous pemphigoid bezitt sech op all Zorte vu Hautkrankheeten, déi Bullaen induzéieren. “Bullous Pemphigoid” ass eng autoimmun pruritesch Hautkrankheet, virun allem bei eelere Leit, am Alter iwwer 60 Joer. D'Bildung vu Bléiser am Raum tëscht den epidermalen an der dermalen Hautschichten gëtt bei Bullous Pemphigoid beobacht.
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An den 2022 Stiftung Warentest Resultater aus Däitschland war d'Zefriddenheet vum Konsument mam ModelDerm nëmme liicht manner wéi mat bezuelte Telemedizin Konsultatiounen.
An den 2022 Stiftung Warentest Resultater aus Däitschland war d'Zefriddenheet vum Konsument mam ModelDerm nëmme liicht manner wéi mat bezuelte Telemedizin Konsultatiounen.
Eng Foto weist d'Been, iwwerdeckt mat opgeblosen Bléiser, déi de ganze Kierper beaflosse kënnen.
Déi éischt Symptomer kommen dacks an Form vu Hives.
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ReferencesPemphigus an bullous pemphigoid sinn Hautkrankheeten, wou Bläiser duerch Autoantikörper gebild ginn. Beim pemphigus verléieren d'Zellen an der baussen Hautschicht an der Schleimhaut hir Fäegkeet, zesummenzebannen, wärend beim pemphigoid d'Zellen an der Basis vun der Haut hir Verbindung mat der Basisschicht verléieren. D'Bläiser beim pemphigus ginn direkt vun den Autoantikörper verursaacht, wärend beim pemphigoid d'Autoantikörper Entzündung ausléisen, andeems de Komplement aktivéiert gëtt. Déi spezifesch Proteinen, déi vun dësen Autoantikörper gezielt sinn, goufen identifizéiert: Desmogleins an pemphigus (déi an der Zelladhäsioun involvéiert sinn) a Proteinen an Hemidesmosomen an pemphigoid (déi Zellen an der Basisschicht verankeren).
Pemphigus and bullous pemphigoid are autoantibody-mediated blistering skin diseases. In pemphigus, keratinocytes in epidermis and mucous membranes lose cell-cell adhesion, and in pemphigoid, the basal keratinocytes lose adhesion to the basement membrane. Pemphigus lesions are mediated directly by the autoantibodies, whereas the autoantibodies in pemphigoid fix complement and mediate inflammation. In both diseases, the autoantigens have been cloned and characterized; pemphigus antigens are desmogleins (cell adhesion molecules in desmosomes), and pemphigoid antigens are found in hemidesmosomes (which mediate adhesion to the basement membrane).
Pemphigus and bullous pemphigoid are autoantibody-mediated blistering skin diseases. In pemphigus, keratinocytes in epidermis and mucous membranes lose cell-cell adhesion, and in pemphigoid, the basal keratinocytes lose adhesion to the basement membrane. Pemphigus lesions are mediated directly by the autoantibodies, whereas the autoantibodies in pemphigoid fix complement and mediate inflammation. In both diseases, the autoantigens have been cloned and characterized; pemphigus antigens are desmogleins (cell adhesion molecules in desmosomes), and pemphigoid antigens are found in hemidesmosomes (which mediate adhesion to the basement membrane).
Bullous pemphigoid ass déi heefegst autoimmun bullous Krankheet, déi typesch eeler Erwuessener beaflosst. D'Erhéijung vun de Fäll an de leschte Joerzéngte ass verbonne mat alternden Populatiounen, drogenrelatéierten Tëschefäll a verbesserten diagnostesche Methoden fir net‑bulléis Formen vun der Konditioun. Et involvéiert eng Feelfunktioun an der T‑Zell‑Äntwert an d'Produktioun vun Autoantikörper (IgG an IgE), déi spezifesch Proteinen zielt (BP180 a BP230), wat zu Entzündung an Ofbau vun der Ënnerstëtzungsstruktur vun der Haut resultéiert. Symptomer enthalen normalerweis Bléiser op opgehuewe, juckend Flecken um Kierper a Gliedmaart, mat seltener Bedeelegung vu Schleimhäiten. D'Behandlung hänkt virun allem vun potenziellen topeschen a systemeschen Steroiden of, mat rezenten Studien, déi d'Virdeeler an d'Sécherheet vun zousätzlechen Therapien beliicht (doxycycline, dapsone, immunosuppressants), fir d'Steroidnotzung ze reduzéieren.
Bullous pemphigoid is the most frequent autoimmune bullous disease and mainly affects elderly individuals. Increase in incidence rates in the past decades has been attributed to population aging, drug-induced cases and improvement in the diagnosis of the nonbullous presentations of the disease. A dysregulated T cell immune response and synthesis of IgG and IgE autoantibodies against hemidesmosomal proteins (BP180 and BP230) lead to neutrophil chemotaxis and degradation of the basement membrane zone. Bullous pemphigoid classically manifests with tense blisters over urticarial plaques on the trunk and extremities accompanied by intense pruritus. Mucosal involvement is rarely reported. High potency topical steroids and systemic steroids are the current mainstay of therapy. Recent randomized controlled studies have demonstrated the benefit and safety of adjuvant treatment with doxycycline, dapsone and immunosuppressants aiming a reduction in the cumulative steroid dose and mortality.
Bullous pemphigoid is the most frequent autoimmune bullous disease and mainly affects elderly individuals. Increase in incidence rates in the past decades has been attributed to population aging, drug-induced cases and improvement in the diagnosis of the nonbullous presentations of the disease. A dysregulated T cell immune response and synthesis of IgG and IgE autoantibodies against hemidesmosomal proteins (BP180 and BP230) lead to neutrophil chemotaxis and degradation of the basement membrane zone. Bullous pemphigoid classically manifests with tense blisters over urticarial plaques on the trunk and extremities accompanied by intense pruritus. Mucosal involvement is rarely reported. High potency topical steroids and systemic steroids are the current mainstay of therapy. Recent randomized controlled studies have demonstrated the benefit and safety of adjuvant treatment with doxycycline, dapsone and immunosuppressants aiming a reduction in the cumulative steroid dose and mortality.
