Bullous pemphigoid - I-Bullous Pemphigoid
I- I-Bullous Pemphigoid (Bullous pemphigoid) ibhekisela kuzo zonke izinhlobo zokukhubazeka kwesikhumba ezibangela ama-bulla. I-"Bullous pemphigoid" yisifo sesikhumba se-autoimmune pruritic ngokukhethekile kubantu asebekhulile, abaneminyaka engaphezu kwengu-60. Ukwakheka kwamabhamuza esikhaleni phakathi kwezingqimba zesikhumba se-epidermal kanye nesikhumba kubonakala ku-bullous pemphigoid.
☆ Emiphumeleni ka-2022 ye-Stiftung Warentest evela eJalimane, ukwaneliseka kwabathengi nge-ModelDerm bekungaphansi kancane kunokuxhumana okukhokhelwayo kwe-telemedicine.
Isithombe esibonisa imilenze embozwe amabhamuza aqhumile, angathinta wonke umzimba.
Izimpawu zokuqala ngezinye izikhathi ziba isifuba.
References
I- Pemphigus kanye ne- bullous pemphigoid yizifo zesikhumba lapho amabhamuza akheka khona ngenxa yama-autoantibodies. Ku- pemphigus , amaseli ongqimba lwesikhumba olungaphandle kanye nolwelwesi lwamafinyila alahlekelwa amandla awo okunamathelana, kuyilapho kokuthi pemphigoid , amaseli angaphansi kwesikhumba alahlekelwa ukuxhumana kwawo nongqimba olungaphansi. Amabhamuza e- pemphigus abangelwa ngokuqondile amasosha omzimba, kuyilapho kokuthi pemphigoid , amasosha omzimba abangela ukuvuvukala ngokwenza kusebenze okuhambisanayo. Amaprotheni athile aqondiswe yilawa ma-autoantibodies akhonjiwe: ama-desmogleins ku- pemphigus (abambe iqhaza ekunamatheleni kweseli) kanye namaphrotheni kuma-hemidesmosomes ku- pemphigoid (okungamaseli abamba izintambo kungqimba olungaphansi) .
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).
I- Bullous pemphigoid yisifo esivame kakhulu se-autoimmune bullous, esihlasela abantu abadala. Ukwenyuka kwezimo emashumini eminyaka amuva nje kuxhunyaniswa nenani labantu abaguga, izehlakalo ezihlobene nezidakamizwa, kanye nezindlela zokuxilonga ezithuthukisiwe zezinhlobo zesimo ezingezona ezesabekayo. Kuhilela ukungasebenzi kahle ekuphenduleni kwe-T cell kanye nokukhiqizwa kwama-autoantibodies (IgG ne-IgE) aqondise amaprotheni athile (BP180 kanye ne-BP230) , okuholela ekuvuvukeni nasekuwohlokeni kwesakhiwo esisekela isikhumba. Izimpawu ngokuvamile zihlanganisa amabhamuza ekuphakameni, amabala alumayo emzimbeni nasezithweni, nokubandakanyeka okungajwayelekile kolwelwesi lwamafinyila. Ukwelashwa ngokuyinhloko kuncike kuma-topical and systemic steroids anamandla, ngocwaningo lwakamuva olugqamisa izinzuzo nokuphepha kwemithi eyengeziwe (doxycycline, dapsone, immunosuppressants) , okuhloswe ngayo ukunciphisa ukusetshenziswa kwama-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.
