Bullous pemphigoid - Bulozni Pemfigoid
Bulozni Pemfigoid (Bullous pemphigoid) se nanaša na kožne bolezni, ki povzročajo bule. Bulozni Pemfigoid je avtoimunska srbeča kožna bolezen, ki se pojavlja predvsem pri starejših ljudeh, starejših od 60 let. Pri buloznem pemfigoidu opazimo nastanek mehurjev v prostoru med epidermalno in dermalno plastjo kože.
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ReferencesPemphigus in bullous pemphigoid sta kožni bolezni, pri katerih nastanejo mehurji zaradi avtoprotiteles. Pri pemphigusu celice v zunanji plasti kože in sluznice izgubijo sposobnost lepljenja, medtem ko pri bullous pemphigoidu celice v dnu kože izgubijo povezavo s spodnjim slojem. Pri pemphigusu pretisni omoti povzročajo neposredno delovanje avtoprotitel, medtem ko pri bullous pemphigoidu avtoprotitela sprožijo vnetje z aktivacijo komplementa. Identificirani so bili specifični proteini, na katere ciljajo ta avtoprotitela: desmogleini (desmogleins) pri pemphigusu (ki so vključeni v celično adhezijo) in proteini v hemidesmosomih pri bullous pemphigoidu (ki celice zasidrajo na spodnjo plast).
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 najpogostejša avtoimunska bulozna bolezen, ki običajno prizadene starejše odrasle. Porast primerov v zadnjih desetletjih je povezan s staranjem prebivalstva, incidencami, povezanimi z drogami, in izboljšanimi diagnostičnimi metodami za nebulozne oblike bolezni. Vključuje okvaro odziva celic T in proizvodnjo avtoprotiteles (IgG in IgE), ki ciljajo na specifične proteine (BP180 in BP230), kar povzroča vnetje in razgradnjo podporne strukture kože. Simptomi običajno vključujejo mehurje na dvignjenih, srbečih lisah na koži in v udih, pri čemer je prizadetost sluznice redka. Zdravljenje se v prvi vrsti opira na močne topikalne in sistemske steroide, nedavne študije pa poudarjajo koristi in varnost dodatnih terapij, kot so doxycycline, dapsone in immunosuppressants, katerih cilj je zmanjšati uporabo steroidov.
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.
