Bullous pemphigoid
Ny Bullous pemphigoid dia manondro ireo karazana aretin-koditra rehetra miteraka omby. Ny "bullous pemphigoid" dia aretin'ny hoditra autoimmune, indrindra indrindra amin'ny olon-dehibe, mihoatra ny 60 taona.
☆ Tamin'ny valin'ny Stiftung Warentest 2022 avy any Alemaina, ny fahafaham-pon'ny mpanjifa amin'ny ModelDerm dia ambany kely noho ny fifampidinihana telemedicine karama.
Sary mampiseho ny tongotra rakotra blister mipoitra, izay mety hisy fiantraikany amin'ny vatana manontolo.
Ny soritr'aretina voalohany dia indraindray miendrika hives.
References
Pemphigus sy bullous pemphigoid dia aretin-koditra izay miforona ny blisters noho ny autoantibodies. Ao amin'ny pemphigus , ny sela ao amin'ny sosona hoditra ivelany sy ny mucous membrane dia very ny fahafahany mifikitra, raha ao amin'ny pemphigoid kosa, ny sela eo am-pototry ny hoditra dia very ny fifandraisany amin'ny sosona ambaniny. Ny blister amin'ny pemphigus dia vokatry ny autoantibodies mivantana, raha ao amin'ny pemphigoid kosa, ny autoantibodies dia miteraka areti-maso amin'ny fampandehanana ny famenoana. Ny proteinina manokana nokendren'ireo autoantibodies ireo dia fantatra: desmogleins ao amin'ny pemphigus (izay tafiditra amin'ny fidiran'ny sela) ary proteinina ao amin'ny hemidesmosomes ao amin'ny pemphigoid (izay mametraka sela amin'ny sosona ambany) .
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 no aretina autoimmune bullous mahazatra indrindra, mazàna mihatra amin'ny olon-dehibe. Ny fiakaran'ny tranga tato anatin'ny folo taona farany dia mifandray amin'ny mponina efa antitra, trangan-javatra mifandraika amin'ny zava-mahadomelina, ary fomba fitiliana nohatsaraina ho an'ny endrika tsy bullous amin'ny toe-javatra. Tafiditra ao anatin'izany ny tsy fahombiazan'ny valin'ny sela T sy ny famokarana autoantibodies (IgG sy IgE) mikendry proteinina manokana (BP180 sy BP230) , ka miteraka fivontosana sy fahapotehan'ny rafitra mpanohana ny hoditra. Ny soritr'aretina dia matetika ahitana blistering amin'ny tasy mitsangana, mangidihidy eo amin'ny vatana sy rantsambatana, miaraka amin'ny fidiran'ny mucous membrane tsy fahita firy. Ny fitsaboana dia miankina amin'ny steroïde topical sy systemic mahery vaika, miaraka amin'ny fanadihadiana vao haingana izay manasongadina ny tombontsoa sy ny fiarovana amin'ny fitsaboana fanampiny (doxycycline, dapsone, immunosuppressants) , mikendry ny hampihenana ny fampiasana 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.
