Bullous pemphigoid - I-Bullous Pemphigoid
I-Bullous Pemphigoid (Bullous pemphigoid) ibhekisa kuzo zonke iintlobo zokuphazamiseka kwesikhumba ezibangela iibulla. “I-Bullous pemphigoid” yi-autoimmune, pruritic isifo solusu ngokukhethekileyo kubantu abadala, abaneminyaka engaphezu kwe-60. Ukubunjwa kwamadyungudyungu phakathi kweenqanaba le-epidermis kunye ne-dermis kubonakala kwi-Bullous Pemphigoid.
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ReferencesPemphigus kunye ne‑bullous pemphigoid zizifo zolusu apho amadyungudyungu enzeka ngenxa ye‑autoantibodies. Kwi‑pemphigus, iiseli ezikumaleko wolusu olungaphandle kunye nenwebu eziphuma emlonyeni zilahlekelwa kukwazi ukunamathela, ngelixa kwi‑pemphigoid, iiseli ezikumazantsi esikhumba ziphulukana nodibaniso lwazo kumaleko angaphantsi. Amadyungudyungu e‑pemphigus enziwa ngokuthe ngqo zizilwa‑buhlungu ezizimelayo, ngelixa kwi‑pemphigoid, ama‑autoantibodies avusa ukudumba ngokuvula i‑epidermis. Iiproteini ezithile ezijoliswe kwezi autoantibodies zichongiwe: i‑desmogleins kwi‑pemphigus (ebandakanyeka kwi‑cell adhesion) kunye nee‑proteini kwi‑hemidesmosomes kwi‑pemphigoid (ezibamba iiseli ze‑anchor kumaleko angaphantsi).
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 sisifo esixhaphakileyo se‑autoimmune, esichaphazela abantu abadala. Ukunyuka kwamatyala kumashumi eminyaka akutshanje kudityaniswa nabantu abalupheleyo, iimeko ezinxulumene neziyobisi, kunye neendlela eziphuculweyo zokuxilonga ezingenakuchazwa njengezinkunzi zesifo. Ibandakanya ukungasebenzi kakuhle kwempendulo ye‑T cell kunye nokuveliswa kwe‑autoantibodies (IgG kunye ne‑IgE) ejolise kwiiprotheni ezithile (BP180 kunye ne‑BP230), okubangela ukuvuvukala kunye nokonakala kwesakhiwo senkxaso yesikhumba. Iimpawu zidla ngokubandakanya amadyungudyungu ekuphakameni, amabala anemibala emnyama emzimbeni nasemilenzeni, kunye nokubandakanyeka okungaqhelekanga kwenwebu. Unyango ngokuyinhloko luxhomekeke kwi‑topical kunye ne‑systemic steroids, kunye nezifundo zakutshanje ezibonisa inzuzo kunye nokhuseleko lonyango olongezelelweyo (doxycycline, dapsone, immunosuppressants), olujoliswe ekunciphiseni ukusetyenziswa kwe‑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.
