Bullous pemphigoid - Pemphigoid Bullous
Ko te Pemphigoid Bullous (Bullous pemphigoid) e tohu ana i nga momo mate kiri katoa e whakapouri ana i nga puru. Ko te "Bullous pemphigoid" he mate kiri pruritic autoimmune e pai ake ana ki nga taangata pakeke, neke atu i te 60 tau. Ko te hanga o te opupu i te waahi i waenga i nga papa kiri epidermal me te kiri ka kitea i roto i te pemphigoid bullous.
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References
Ko te Pemphigus me te bullous pemphigoid he mate kiri ka puta te pupuhi na te autoantibodies. I roto i te pemphigus , ka ngaro te kaha o nga pūtau o te paparanga kiri o waho me nga kiri mucous ki te piri tahi, i te pemphigoid , ka ngaro te hononga o nga pūtau kei te take o te kiri ki te paparanga o raro. Ko nga pupuhi o te pemphigus na nga autoantibodies, i te pemphigoid , ko nga autoantibodies ka puta te mumura ma te whakahohe i te taapiri. Ko nga pūmua motuhake e whaaia ana e enei autoantibodies kua tautuhia: desmogleins i roto i te pemphigus (e whai waahi ana ki te adhesion pūtau) me nga pūmua i roto i te hemidesmosomes i roto i te pemphigoid (he punga nga pūtau ki te paparanga o raro) .
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).
Ko te Bullous pemphigoid te mate autoimmune bullous tino noa, e pa ana ki nga pakeke pakeke. Ko te pikinga o nga keehi i roto i nga tekau tau tata nei e hono ana ki nga taupori taipakeke, nga maiki e pa ana ki te tarukino, me te pai ake o nga tikanga tātaritanga mo nga momo mate kore-bullous. Kei roto i te kino o te whakautu T cell me te hanga o nga autoantibodies (IgG me IgE) e aro ana ki nga pūmua motuhake (BP180 me BP230) , ka puta te mumura me te pakaru o te hanganga tautoko o te kiri. Ko te nuinga o nga tohu ko te pupuhi i runga i nga papa kua piki ake, i te patitoka ki te tinana me nga peka, me te onge o te uru mai o nga kiriuhi mucous. Ko te nuinga o te maimoatanga e whakawhirinaki ana ki nga steroids totika me te punaha, me nga rangahau tata e whakaatu ana i nga painga me te haumaru o nga rongoa taapiri (doxycycline, dapsone, immunosuppressants) , e whai ana ki te whakaiti i te whakamahi steroid.
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.
