Bullous pemphigoid - Bulozni Pemfigoid
Bulozni Pemfigoid (Bullous pemphigoid) odnosi se na sve vrste kožnih poremećaja koji uzrokuju bule. "Bulozni pemfigoid" je autoimuna kožna bolest sa svrbežom, ponajprije u starijih ljudi, starijih od 60 godina. Kod buloznog pemfigoida uočava se stvaranje mjehurića u prostoru između epidermalnog i dermalnog sloja kože.
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Pemphigus i bullous pemphigoid su kožne bolesti kod kojih se stvaraju mjehurići zbog autoantitijela. U pemphigus , stanice u vanjskom sloju kože i sluznici gube svoju sposobnost lijepljenja, dok u pemphigoid , stanice u dnu kože gube vezu s donjim slojem. Mjehuriće kod pemphigus izravno uzrokuju autoantitijela, dok kod pemphigoid autoantitijela pokreću upalu aktiviranjem komplementa. Identificirani su specifični proteini na koje ciljaju ova autoantitijela: dezmogleini u pemphigus (koji su uključeni u staničnu adheziju) i proteini u hemidesmosomima u pemphigoid (koji usidre stanice za sloj ispod) .
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 je najčešća autoimuna bulozna bolest, koja obično pogađa starije osobe. Porast slučajeva tijekom posljednjih desetljeća povezan je sa starenjem populacije, incidentima povezanim s drogama i poboljšanim dijagnostičkim metodama za nebulozne oblike stanja. Uključuje kvar u odgovoru T stanica i proizvodnju autoantitijela (IgG i IgE) usmjerenih na specifične proteine (BP180 i BP230) , što rezultira upalom i razgradnjom potporne strukture kože. Simptomi obično uključuju mjehuriće na uzdignutim mrljama koje svrbe na tijelu i udovima, s rijetkim zahvaćanjem sluznice. Liječenje se primarno oslanja na snažne topikalne i sistemske steroide, a nedavne studije ističu dobrobiti i sigurnost dodatnih terapija kao što je doxycycline, dapsone, and immunosuppressants , usmjerenih na smanjenje upotrebe steroida.
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.
