Bullous pemphigoid - Penfigoyid Boulous
Pemfigoid boulè (Bullous pemphigoid) refere tout kalite maladi po ki pwovoke boulè. "Bullous pemphigoid" se yon maladi po otoiminitè ki gratèl preferansyèlman nan moun ki pi gran, ki gen plis pase 60. Se fòmasyon nan ti boul nan espas ki genyen ant kouch epidermis ak dermis ki obsève nan pemfigoid boulè.
☆ Nan rezilta Stiftung Warentest 2022 ki soti nan Almay, satisfaksyon konsomatè yo ak ModelDerm te sèlman yon ti kras pi ba pase ak konsiltasyon telemedsin peye.
Yon foto ki montre janm kouvri nan ti anpoul eklate, ki ka afekte tout kò a.
Sentòm inisyal yo pafwa nan fòm itikè.
relevance score : -100.0%
ReferencesPemphigus ak bullous pemphigoid se maladi po kote ti boul (blisters) fòme akòz otoantikò. Nan pemphigus, selil ki nan kouch ekstèn po a ak manbràn mikez yo pèdi kapasite yo pou yo kole ansanm, pandan y ap nan pemphigoid, selil ki nan baz po a pèdi koneksyon yo ak kouch ki kache a. Boul yo (blisters) nan pemphigus yo lakòz dirèkteman pa otoantikò yo, pandan y ap 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 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 (pemfigoïde bulleux) se maladi otoiminitè ki pi komen, anjeneral ki afekte granmoun aje yo. Ogmantasyon nan ka yo nan dènye deseni yo lye ak popilasyon ki aje, ensidan ki gen rapò ak dwòg, ak amelyorasyon metòd dyagnostik pou fòm ki pa boulous nan kondisyon an. Li enplike yon fonksyon byen nan repons selil T ak pwodiksyon an nan otoantikò (IgG ak IgE) ki vize pwoteyin espesifik (BP180 ak BP230), sa ki lakòz enflamasyon ak pann nan estrikti sipò po a. Sentòm yo anjeneral gen ladan boul ki tache (blisters), plak urtikè (urticarial plaques) sou kò a ak branch, ak patisipasyon ra nan manbràn mikoz (mucosal involvement). Tretman prensipalman depann sou estewoyid ki pisan topikal (topical) ak sistemik, ak etid resan yo mete aksan sou benefis yo ak sekirite nan terapi adisyonèl (doxycycline, dapsone, immunosuppressants), ki vize a 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.
