Bullous pemphigoid - Pemfîgoîdê Bullous
Pemfîgoîdê Bullous (Bullous pemphigoid) her cure nexweşiyên çerm ên ku bullan çêdikin vedibêje. "Bullous pemphigoid" nexweşiyek çermê ya otoîmmune ye ku bi taybetî di mirovên kal û pîr de, ji 60 salî mezintir e. Çêbûna bilbilan di valahiya navbera qatên çermê epidermal û çerm de di pemphigoîdê bulous de tê dîtin.
☆ Di encamên 2022-an de Stiftung Warentest ji Almanyayê, razîbûna xerîdar bi ModelDerm tenê ji şêwirmendên telemedicine drav hindik hindiktir bû.
Wêneyek ku lingên xwe di bilbilên pêçandî de nîşan dide, ku dikare bandorê li tevahiya laş bike.
Nîşaneyên destpêkê carinan di forma hêlînê de ne.
References
Pemphigus û bullous pemphigoid nexweşiyên çerm in ku ji ber otoantîbodîtan bilbil çêdibin. Di pemphigus de, xaneyên li qata çermê ya derve û parzûnên mukozê şiyana xwe ya ku bi hev ve girêdidin winda dikin, lê di pemphigoid de, şaneyên li binê çerm girêdana xwe ya bi tebeqeya binî re winda dikin. Pûçikên pemphigus rasterast ji hêla otoantîbodîsan ve têne çêkirin, dema ku di pemphigoid de, otoantîbodî bi çalakkirina temamkerê iltîhaba çêdikin. Proteînên taybetî yên ku ji hêla van otoantîbodîyan ve têne armanc kirin hatine nas kirin: desmogleînên di pemphigus de (yên ku di girêdana şaneyê de beşdar in) û proteînên di hemidesmosomes de di pemphigoid de (ku hucreyan li qata binî ve girêdidin) .
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 nexweşiya bulousê ya otoîmmune ya herî gelemperî ye, bi gelemperî mezinan bandor dike. Zêdebûna bûyeran di van dehsalên dawî de bi pîrbûna nifûsê, bûyerên bi narkotîkê ve, û baştirkirina rêbazên tespîtkirinê yên ji bo formên ne-bulous ên rewşê ve girêdayî ye. Ew di bersiva hucreya T de xeletiyek heye û hilberîna otoantîbodîyan (IgG û IgE) ku proteînên taybetî (BP180 û BP230) dikin hedef, di encamê de iltîhaba û têkçûna avahiya piştgirî ya çerm pêk tê. Nîşanan bi gelemperî di nav pêlên bilindkirî, xişkirî yên li ser laş û lingan de, bi tevlêbûna membranên mukozê yên kêm kêm pêk tê. Dermankirin di serî de xwe dispêre steroîdên topkî û pergalî yên bi hêz, digel lêkolînên vê dawiyê feyde û ewlehiya dermanên zêde (doxycycline, dapsone, immunosuppressants) , ku bi mebesta kêmkirina karanîna steroîdan ronî dikin.
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.
