Bullous pemphigoid - Bulozni Pemfigoid
Bulozni Pemfigoid (Bullous pemphigoid) odnosi se na sve vrste kožnih poremećaja koji izazivaju bule. "Bulozni pemfigoid" je autoimuno oboljenje kože sa svrabom prvenstveno kod starijih osoba, starijih od 60 godina. Kod buloznog pemfigoida uočava se stvaranje plikova u prostoru između epidermalnog i dermalnog sloja kože.
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ReferencesPemfigus (pemphigus) i bullous pemfigoid (bullous pemphigoid) su kožne bolesti kod kojih se stvaraju plikovi zbog autoantitijela. U pemfigusu, ćelije u spoljašnjem sloju kože i sluzokože gube svoju sposobnost da se drže zajedno, dok u pemfigoidu ćelije u bazi kože gube vezu sa donjim slojem. Plikovi pemfigusa su uzrokovani direktno autoantitijelima, dok u pemfigoidu autoantitijela izazivaju upalu aktiviranjem komplementa. Identificirani su specifični proteini na koje ciljaju ova autoantitijela: desmogleini u pemfigusu (koji su uključeni u ćelijsku adheziju) i proteini u hemidesmosomima u pemfigoidu (koji pričvršćuju ćelije 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 odrasle osobe. Porast slučajeva u posljednjih nekoliko desetljeća povezan je sa starenjem populacije, incidentima povezanim s drogom i poboljšanim dijagnostičkim metodama za ne‑bulozne oblike stanja. Uključuje kvar u T‑ćelijskom odgovoru i proizvodnju autoantitela (IgG i IgE) usmjerenih na specifične proteine (BP180 i BP230), što rezultira upalom i slomom potporne strukture kože. Simptomi obično uključuju pojavu plikova na podignutim mrljama koje svrbe na tijelu i udovima, s rijetkim zahvaćanjem sluzokože. Liječenje se prvenstveno oslanja na moćne lokalne i sistemske steroide, a nedavne studije naglašavaju prednosti i sigurnost dodatnih terapija kao što su doksiciklin (doxycycline), dapsone i imunosupresivi (immunosuppressants), čiji je cilj 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.
