Bullous pemphigoid - Bulleuse Pemfigoïed
Bulleuse Pemfigoïed (Bullous pemphigoid) verwys na alle soorte velafwykings wat bullaes veroorsaak. "Bullous pemfigoïd" is 'n outo-immuun pruritiese velsiekte by voorkeur in ouer mense, ouer as 60. Die vorming van blase in die spasie tussen die epidermale en dermale vellae word waargeneem in bullose pemfigoïed.
☆ In die 2022 Stiftung Warentest-resultate van Duitsland was verbruikerstevredenheid met ModelDerm net effens laer as met betaalde telemedisyne-konsultasies.
'n Foto wat bene toon wat met blase bedek is, wat die hele liggaam kan affekteer.
Die aanvanklike simptome is soms in die vorm van korwe.
References
Pemphigus en bullous pemphigoid is velsiektes waar blase vorm as gevolg van outo-teenliggaampies. In pemphigus verloor selle in die buitenste vellaag en slymvliese hul vermoë om aan mekaar te kleef, terwyl in pemphigoid selle aan die basis van die vel hul verbinding met die onderliggende laag verloor. Die blase van pemphigus word direk deur die outo-teenliggaampies veroorsaak, terwyl in pemphigoid die outo-teenliggaampies ontsteking veroorsaak deur komplement te aktiveer. Die spesifieke proteïene wat deur hierdie outo-teenliggaampies geteiken is, is geïdentifiseer: desmogleins in pemphigus (wat betrokke is by seladhesie) en proteïene in hemidesmosome in pemphigoid (wat selle aan die onderliggende laag anker) .
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 is die mees algemene outo-immuun bullose siekte, wat gewoonlik ouer volwassenes affekteer. Die toename in gevalle oor die afgelope dekades is gekoppel aan veroudering van bevolkings, dwelmverwante voorvalle en verbeterde diagnostiese metodes vir nie-bulle vorme van die toestand. Dit behels 'n wanfunksie in T-selreaksie en die produksie van outo-teenliggaampies (IgG en IgE) wat spesifieke proteïene (BP180 en BP230) teiken, wat lei tot inflammasie en afbreek van die vel se ondersteunende struktuur. Simptome sluit gewoonlik blase op verhewe, jeukerige kolle op die liggaam en ledemate in, met seldsame betrokkenheid van slymvliese. Behandeling berus hoofsaaklik op kragtige aktuele en sistemiese steroïede, met onlangse studies wat die voordele en veiligheid van addisionele terapieë beklemtoon (doxycycline, dapsone, immunosuppressants) , wat daarop gemik is om steroïedgebruik te verminder.
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.
