Bullous pemphigoid - Penfigoyid Boulous
Penfigoyid Boulous (Bullous pemphigoid) refere a tout kalite maladi po ki pwovoke bulyè. “Bullous pemphigoid” se yon maladi po otoiminitè ki fè gratèl, prensipalman nan moun ki pi gran, ki gen plis pase 60 an. Se fòmasyon ti anpoul nan espas ki genyen ant kouch po epidérme a ak po ki pi fon yo, ki obsève nan Penfigoyid Boulous.
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ReferencesPemphigus ak bullous pemphigoid se maladi po kote ti anpoul fòme akoz otoantikò. Nan pemphigus, selil ki nan kouch ekstèn po a ak manbràn mikroz yo pèdi kapasite pou yo kole ansanm; pandan ke nan pemphigoid, selil ki nan baz po a pèdi koneksyon ak kouch ki kache a. Anpoul ki parèt nan pemphigus yo koze dirèkteman pa otoantikò, tandiske nan pemphigoid, otoantikò yo deklanche enflamasyon lè yo aktive konpleman. Pwoteyin espesifik ki vize pa otoantikò sa yo te idantifye: desmoglein nan pemphigus (ki patisipe nan adezyon selilè) ak pwoteyin nan hemidesmosomes nan pemphigoid (ki jete selil yo soti nan kouch ki kache a).
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 se maladi boulous otoiminitè ki pi komen an, anjeneral ki afekte granmoun aje yo. Ogmantasyon kantite ka yo nan dènye deseni a lye ak popilasyon ki ap aje, ak ensidan ki gen rapò ak dwòg, epi ak amelyorasyon metòd dyagnostik pou fòm ki pa boulous nan kondisyon an. Li enplike yon fonksyonman anòmal nan repons selil T ak pwodiksyon otoantikò (IgG ak IgE) ki vize pwoteyin espesifik (BP180 ak BP230), sa ki lakòz enflamasyon ak domaj nan estrikti sipò po a. Sentòm yo anjeneral gen ladan blesi sou po, plak ki grate sou kò a ak branch, epi patisipasyon ra nan manbràn mukozal. Tretman prensipalman depann sou estewoyid ki gen fòs, sou fòm topik ak sistemik, epi etid resan yo mete aksan sou benefis ak sekirite terapi adisyonèl (doxycycline, dapsone, immunosuppressants), ki vize diminye itilizasyon estewoyid.
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.
