Bullous pemphigoid - Bulloosne Pemfigoid
Bulloosne Pemfigoid (Bullous pemphigoid) viitab igasugustele nahahaigustele, mis kutsuvad esile bulla. Bulloosne pemfigoid on autoimmuunne sügelev nahahaigus, mis esineb peamiselt vanematel, üle 60-aastastel inimestel. Bulloosse pemfigoidi puhul on täheldatud villide teket epidermise ja dermaalse nahakihi vahel.
☆ Saksamaa 2022. aasta Stiftung Warentesti tulemustes oli tarbijate rahulolu ModelDermiga vaid veidi madalam kui tasuliste telemeditsiini konsultatsioonidega.
Foto, millel on jalad kaetud villidega, mis võivad mõjutada kogu keha.
Esialgsed sümptomid on mõnikord nõgestõbi.
References
Pemphigus ja bullous pemphigoid on nahahaigused, kus autoantikehade toimel tekivad villid. Kui pemphigus kaotavad naha välimise kihi ja limaskestade rakud oma võime kokku kleepuda, samas kui pemphigoid puhul kaotavad nahapõhja rakud ühenduse aluskihiga. Pemphigus villid on otseselt põhjustatud autoantikehadest, samas kui pemphigoid korral käivitavad autoantikehad põletiku, aktiveerides komplemendi. Nende autoantikehade sihtmärgiks olevad spetsiifilised valgud on tuvastatud: pemphigus desmogleiinid (mis on seotud raku adhesiooniga) ja valgud hemidesmosoomides pemphigoid (mis ankurdavad rakud aluskihi külge) .
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 on kõige levinum autoimmuunne bulloosne haigus, mis mõjutab tavaliselt vanemaid täiskasvanuid. Juhtumite arvu suurenemine viimastel aastakümnetel on seotud elanikkonna vananemise, uimastitega seotud vahejuhtumite ja paranenud diagnoosimeetoditega haigusseisundi mitte-bulloossete vormide puhul. See hõlmab talitlushäireid T-raku vastuses ja spetsiifilistele valkudele (BP180 ja BP230) suunatud autoantikehade (IgG ja IgE) tootmist, mille tulemuseks on põletik ja naha tugistruktuuri lagunemine. Sümptomiteks on tavaliselt villid kerkinud, sügelevad laigud kehal ja jäsemetel, harva esinevad limaskestad. Ravi põhineb peamiselt tugevatoimelistel paiksetel ja süsteemsetel steroididel, hiljutised uuringud rõhutavad steroidide kasutamise vähendamisele suunatud täiendavate ravimeetodite, nagu doxycycline, dapsone, and immunosuppressants , eeliseid ja ohutust.
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.
