Bullous pemphigoid - Pemphigoid Bullous
Tagraíonn Pemphigoid Bullous (Bullous pemphigoid) do gach cineál neamhoird craicinn a chothaíonn bullaes. Is galar craiceann pruritic autoimmune é "bullous pemphigoid" i ndaoine scothaosta, os cionn 60 bliain d'aois. Breathnaítear foirmiú blisters sa spás idir na sraitheanna craiceann epidermal agus deirmeach i pemphigoid bullous.
☆ I dtorthaí 2022 Stiftung Warentest ón nGearmáin, ní raibh sástacht na dtomhaltóirí le ModelDerm ach beagán níos ísle ná mar a bhí le comhairliúchán teileamhíochaine íoctha.
Grianghraf a thaispeánann na cosa clúdaithe le blisters popped, a d'fhéadfadh cur isteach ar an gcorp ar fad.
Uaireanta bíonn na hairíonna tosaigh i bhfoirm hives.
References
Is galair craicinn iad Pemphigus agus bullous pemphigoid ina gcruthaítear blisters de bharr uath-antasubstaintí. I pemphigus , cailleann cealla sa chiseal seachtrach craiceann agus seicní múcasacha a gcumas cloí le chéile, agus i pemphigoid , caillfidh cealla ag bun an chraiceann a nasc leis an gciseal bunúsach. Is iad na uath-antasubstaintí is cúis go díreach leis na blisters de pemphigus , agus i pemphigoid , spreagann na n-uath-antasubstaintí athlasadh trí chomhlánú a ghníomhachtú. Sainaithníodh na próitéiní sonracha a ndírítear orthu ag na n-uath-antasubstaintí seo: desmogleins i pemphigus (a bhfuil baint acu le greamaitheacht cille) agus próitéiní i hemidesmosomes i pemphigoid (a cheanglaíonn cealla leis an mbunchiseal) .
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).
Is é Bullous pemphigoid an galar tarbh uath-imdhíonta is coitianta, a théann i bhfeidhm ar dhaoine fásta go hiondúil. Tá an t-ardú ar chásanna le blianta beaga anuas nasctha le daonraí atá ag dul in aois, teagmhais a bhaineann le drugaí, agus modhanna diagnóiseacha feabhsaithe maidir le foirmeacha neamhbhuartha den riocht. Baineann sé le mífheidhmiú i bhfreagra cille T agus táirgeadh autoantibodies (IgG agus IgE) a dhíríonn ar phróitéiní sonracha (BP180 agus BP230) , rud a fhágann athlasadh agus briseadh síos ar struchtúr tacaíochta an chraiceann. Áirítear ar na comharthaí de ghnáth blistering ar phaistí ardaithe, itchy ar an gcorp agus géaga, le baint annamh na seicní múcasacha. Braitheann an chóireáil go príomha ar stéaróidí tráthúla agus sistéamacha cumhachtacha, agus léirigh staidéir le déanaí na buntáistí agus an tsábháilteacht a bhaineann le teiripí breise (doxycycline, dapsone, immunosuppressants) , atá dírithe ar úsáid stéaróideach a laghdú.
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.
