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 fa'avaeina o ma'i pa'u i le va i le epidermis ma le dermis o lo'o matauina i bullous pemphigoid.
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ReferencesPemphigus 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 epidermis (epidermis) ma mucous membrane e leiloa lo latou malosi e pipii faatasi, ae i bullous pemphigoid (bullous pemphigoid), o sela i le basal keratinocytes (basal keratinocytes) ua leiloa lo latou sootaga ma le basement membrane (basement membrane). O ma'i pa'u o pemphigus e mafua sa'o mai i le autoantibodies, ae i bullous pemphigoid (bullous 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 (desmogleins) (lea e a'afia i le fa'apipi'i o sela) ma polotini i hemidesmosomes i bullous pemphigoid (bullous pemphigoid) (lea e taula ai sela i le basement membrane (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).
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 ma'i o le pa'u e fa'atuina ai bullae. E masani ona afaina tagata matutua, e sili ona i'uga i tagata o lo'o matua'i 60 tausaga. O le fa'afitauli e tupu i le va o le epidermis ma le dermis. O le lele o le T‑cell ma le gaosiga o le auto‑antibodies (IgG ma le IgE) i le polotini fa'apitoa (BP180 ma le BP230) e mafua ai le fa'ata'ita'i o le pa'u ma le fa'aleagaina o le basement membrane. O le ma'i e masani ona faʻaalia i le fa'ata'ita'i o le pa'u malosi i luga o le pa'u urticarial i le tino ma le vae, fa'atasi ai ma le pruritus malosi. O le auai i le mucous membranes e itiiti ona fa'ailoa. O le togafitiga autu o le faʻaaogaina o le steroid maualuga i le fata ma le systemic steroid, ma le fa'aogaina o le doxycycline, dapsone, immunosuppressants e fesoasoani i le fa'aitiitia o le dose 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.
