Bullous pemphigoid - Pemphigoid Bullous
Tha Pemphigoid Bullous (Bullous pemphigoid) a’ toirt iomradh air a h-uile seòrsa eas-òrdugh craiceann a bhios ag adhbhrachadh bullaes. Tha "bullous pemphigoid" na ghalar craiceann fèin-dìonach pruritic ann an seann daoine, nas sine na 60. Thathas a’ cumail sùil air cruthachadh blisters anns an àite eadar na sreathan craiceann epidermal agus dermal ann am pemphigoid bullous.
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References
Is e galairean craiceann a th’ ann an Pemphigus agus bullous pemphigoid far a bheil blisters a’ cruthachadh mar thoradh air autoantibodies. Ann an pemphigus , bidh ceallan ann an còmhdach a’ chraicinn a-muigh agus fileagan mucous a’ call an comas cumail ri chèile, agus ann an pemphigoid , bidh ceallan aig bonn a’ chraicinn a’ call an ceangal ris an t-sreath bhunasach. Tha na blisters de pemphigus air an adhbhrachadh gu dìreach leis na autoantibodies, agus ann an pemphigoid , bidh na h-auto-antibodies a 'brosnachadh sèid le bhith a' cur an gnìomh co-fhillte. Chaidh na pròtanan sònraichte a tha air an cuimseachadh leis na autoantibodies sin a chomharrachadh: desmogleins ann an pemphigus (a tha an sàs ann an gèilleadh cealla) agus pròtanan ann an hemidesmosomes ann an pemphigoid (a tha a’ ceangal cheallan ris an t-sreath bhunasach) .
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 e Bullous pemphigoid an galar tarbh fèin-dìonach as cumanta, mar as trice a’ toirt buaidh air inbhich nas sine. Tha an àrdachadh ann an cùisean thar nan deicheadan mu dheireadh co-cheangailte ri àireamhan a tha a’ fàs nas sine, tachartasan co-cheangailte ri drogaichean, agus dòighean sgrùdaidh nas fheàrr airson cruthan neo-tharbhach den t-suidheachadh. Tha e a’ toirt a-steach mì-ghnàthachadh ann am freagairt cealla T agus cinneasachadh autoantibodies (IgG agus IgE) ag amas air pròtanan sònraichte (BP180 agus BP230) , a’ leantainn gu sèid agus briseadh sìos air structar taiceil a’ chraicinn. Mar as trice bidh comharran a’ toirt a-steach sèididh air badan àrdaichte, tachasach air a’ bhodhaig agus na buill-bodhaig, le com-pàirt tearc de mhucous membranes. Tha làimhseachadh gu sònraichte an urra ri steroids gnàthach is siostamach cumhachdach, le sgrùdaidhean o chionn ghoirid a’ soilleireachadh buannachdan agus sàbhailteachd leigheasan a bharrachd (doxycycline, dapsone, immunosuppressants) , ag amas air cleachdadh steroid a lughdachadh.
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.
