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 esine-pruritic ngokukhethekile kubantu asebekhulile, abaneminyaka engaphezu kwengu-60. Ukubunjwa kwamabhamuza esikhaleni phakathi kwezingqimba zesikhumba se-epidermal kanye ne-dermal kubonakala ku-bullous pemphigoid.
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ReferencesI-Pemphigus kanye ne-bullous pemphigoid yizifo zesikhumba lapho amabhamuza akheka khona ngenxa yama-autoantibodies. Ku-pemphigus, amaseli e-epidermis kanye ne-dermis alahlekelwa amandla awo okunamathelana, kuyilapho ku-pemphigoid, amaseli angaphansi kwesikhumba alahlekelwa ukuxhumana kwawo kwi-dermis. Amabhamuza e-pemphigus abangelwa ngokuqondile amasosha omzimba, kuyilapho ku-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 (okubamba i-hemidesmosomes ku-dermis).
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 e‑urticaria (urticarial plaques) emzimbeni nasezithweni, ukubandakanyeka kwethambo lokuhlinza (mucosal involvement) kuyinto evame ukubonwa kancane. 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.
