Bullous pemphigoid
Bullous pemphigoid e fa'asino i ituaiga uma o fa'afitauli o le pa'u e fa'aoso ai pulu. "Bullous pemphigoid" o se autoimmune pruritic maʻi paʻu e sili atu i tagata matutua, matutua i luga atu o le 60. O le faʻavaeina o maʻi paʻu i le va o le epidermal ma le paʻu paʻu o loʻo matauina i bullous pemphigoid.
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References
Pemphigus ma bullous pemphigoid o fa'ama'i pa'u ia e ma'i pa'u ona o le autoantibodies. I le pemphigus , o sela i le pito i fafo o le pa'u o le pa'u ma mucous membrane e leiloa lo latou malosi e pipii faatasi, ae i pemphigoid , o sela i le pito i lalo o le paʻu ua leiloa lo latou sootaga ma le vaega o loʻo i lalo. O ma'i pa'u o pemphigus e mafua sa'o mai i le autoantibodies, ae i pemphigoid , o le autoantibodies e fa'aosoina ai le mumū e ala i le fa'agaoioia o le complement. O polotini fa'apitoa e fa'atatau i nei autoantibodies ua fa'ailoaina: desmogleins i pemphigus (lea e a'afia i le fa'apipi'i o sela) ma polotini i hemidesmosomes i pemphigoid (lea e taula ai sela i le vaega pito i lalo) .
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 o le fa'ama'i fa'ama'i e sili ona taatele, e masani ona a'afia ai tagata matutua. O le si'itia o mataupu i le tele o tausaga talu ai e feso'ota'i ma le faitau aofa'i o tagata matutua, fa'alavelave fa'atatau i fualaau fa'asaina, ma fa'aleleia atili metotia fa'ata'ita'i mo fa'ama'i e le o le bullous. E a'afia ai se fa'aletonu ile tali ole T cell ma le gaosiga ole autoantibodies (IgG ma le IgE) e fa'atatau i polotini fa'apitoa (BP180 ma le BP230) , e mafua ai le mumū ma le malepelepe o le fausaga lagolago o le pa'u. O auga e masani ona aofia ai le ma'i pipii i luga, magese pa'u i luga o le tino ma vae, ma e seasea aafia ai le mucous membranes. Togafitiga e faʻalagolago i luga o le malosi ma le faʻaogaina o mea faʻamaʻi, faʻatasi ai ma suʻesuʻega talu ai nei o loʻo faʻamaonia ai aoga ma le saogalemu o togafitiga faʻaopoopo (doxycycline, dapsone, immunosuppressants) , e faʻatatau i le faʻaitiitia o le faʻaogaina o le 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.
