Bullous pemphigoid
Bullous pemphigoid ferwiist alle soarten hûd oandwaning dy't induce bullaes. "Bullous pemphigoid" is in autoimmune prurityske hûdsykte by foarkar yn âldere minsken, âlder as 60. De foarming fan blieren yn 'e romte tusken de epidermale en dermale hûdlagen wurdt waarnommen yn bullous pemphigoid.
☆ Yn 'e 2022 Stiftung Warentest-resultaten út Dútslân wie de konsuminttefredenheid mei ModelDerm mar wat leger dan mei betelle telemedisynkonsultaasjes.
In foto dy't skonken toant bedekt mei blierren, dy't it hiele lichem kinne beynfloedzje.
De earste symptomen binne soms yn 'e foarm fan hives.
References
Pemphigus en bullous pemphigoid binne hûdsykten dêr't blieren foarmje troch autoantistoffen. Yn pemphigus ferlieze sellen yn 'e bûtenste hûdlaach en slijmvliezen har fermogen om byinoar te plakjen, wylst yn pemphigoid sellen oan 'e basis fan 'e hûd har ferbining ferlieze mei de ûnderlizzende laach. De blieren fan pemphigus wurde direkt feroarsake troch de autoantistoffen, wylst yn pemphigoid de autoantylders ûntstekking triggerje troch it aktivearjen fan komplement. De spesifike aaiwiten dy't rjochte binne troch dizze autoantistoffen binne identifisearre: desmogleins yn pemphigus (dy't belutsen binne by seladhesion) en aaiwiten yn hemidesmosomen yn pemphigoid (dy't sellen anker oan 'e ûnderlizzende laach) .
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 de meast foarkommende autoimmune bullous sykte, dy't typysk âldere folwoeksenen beynfloedet. De opkomst yn gefallen oer de lêste desennia is keppele oan fergrizing populaasjes, drugs-relatearre ynsidinten, en ferbettere diagnostyske metoaden foar net-bullous foarmen fan de tastân. It giet om in misfunksje yn T-selreaksje en de produksje fan autoantistoffen (IgG en IgE) rjochte op spesifike aaiwiten (BP180 en BP230) , wat resulteart yn ûntstekking en ôfbraak fan 'e stypjende struktuer fan' e hûd. Symptomen omfetsje gewoanlik blieren op ferhege, jeukende plakken op it lichem en de ledematen, mei seldsume belutsenens fan slijmvliezen. Behanneling fertrout primêr op krêftige topikale en systemyske steroïden, mei resinte stúdzjes dy't de foardielen en feiligens fan ekstra terapyen markearje (doxycycline, dapsone, immunosuppressants) , rjochte op it ferminderjen fan steroidgebrûk.
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.
