Bullous pemphigoid - Pemphigoid Tarwol
Mae Pemphigoid Tarwol (Bullous pemphigoid) yn cyfeirio at bob math o anhwylderau croen sy'n achosi bulla. Mae "pemphigoid tarw" yn glefyd croen pruritig awtoimiwn sy'n cael ei ffafrio mewn pobl hŷn, dros 60 oed. Gwelir ffurfio pothelli yn y gofod rhwng haenau croen epidermaidd a dermol mewn pemphigoid tarw.
☆ Yng nghanlyniadau Stiftung Warentest 2022 o’r Almaen, roedd boddhad defnyddwyr â ModelDerm ond ychydig yn is nag ymgynghoriadau telefeddygaeth taledig.
Llun yn dangos coesau wedi'u gorchuddio â phothelli popiog, a all effeithio ar y corff cyfan.
Mae'r symptomau cychwynnol weithiau ar ffurf cychod gwenyn.
References
Mae Pemphigus a bullous pemphigoid yn glefydau croen lle mae pothelli'n ffurfio oherwydd awto-wrthgyrff. Yn pemphigus , mae celloedd yn haen allanol y croen a philenni mwcaidd yn colli eu gallu i gadw at ei gilydd, tra yn pemphigoid , mae celloedd ar waelod y croen yn colli eu cysylltiad â'r haen waelodol. Mae pothelli pemphigus yn cael eu hachosi'n uniongyrchol gan yr autoantibodies, tra yn pemphigoid , mae'r autoantibodies yn sbarduno llid trwy actifadu cyflenwad. Mae'r proteinau penodol a dargedir gan yr awto-wrthgyrff hyn wedi'u nodi: desmogleins yn pemphigus (sy'n ymwneud ag adlyniad celloedd) a phroteinau mewn hemidesmosomau yn pemphigoid (sy'n angori celloedd i'r haen waelodol) .
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 yw'r clefyd tarw awtoimiwn mwyaf cyffredin, sy'n effeithio ar oedolion hŷn fel arfer. Mae’r cynnydd mewn achosion dros y degawdau diwethaf yn gysylltiedig â phoblogaethau sy’n heneiddio, digwyddiadau’n ymwneud â chyffuriau, a gwell dulliau diagnostig ar gyfer ffurfiau di-fwlch o’r cyflwr. Mae'n cynnwys camweithio mewn ymateb celloedd T a chynhyrchu awtogffynnau (IgG ac IgE) sy'n targedu proteinau penodol (BP180 a BP230) , gan arwain at lid a dadansoddiad o strwythur cynhaliol y croen. Mae'r symptomau fel arfer yn cynnwys pothellu ar ddarnau uchel sy'n cosi ar y corff a'r aelodau, gydag ymglymiad prin o bilenni mwcaidd. Mae triniaeth yn dibynnu'n bennaf ar steroidau cyfoes a systemig cryf, gydag astudiaethau diweddar yn amlygu manteision a diogelwch therapïau ychwanegol (doxycycline, dapsone, immunosuppressants) , gyda'r nod o leihau'r defnydd o steroidau.
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.
