Bullous pemphigoid - Bulløs Pemphigoid
Bulløs Pemphigoid (Bullous pemphigoid) refererer til alle slags hudlidelser, der fremkalder bullae. Bulløs Pemphigoid er en autoimmun, kløende hudsygdom, primært hos personer over 60 år. Dannelsen af blærer i mellemrummet mellem de epidermale og dermale hudlag observeres ved Bulløs Pemphigoid.
☆ I 2022 Stiftung Warentest-resultaterne fra Tyskland var forbrugernes tilfredshed med ModelDerm kun lidt lavere end med betalte telemedicinske konsultationer.
Et billede, der viser ben dækket af sprængte vabler, som kan påvirke hele kroppen.
De første symptomer er nogle gange i form af nældefeber.
relevance score : -100.0%
ReferencesPemphigus og bullous pemphigoid er hudsygdomme, hvor der dannes blærer på grund af autoantistoffer. I pemphigus mister cellerne i det ydre hudlag og på slimhinderne deres evne til at holde sammen, mens cellerne i hudens basallag i pemphigoid mister deres forbindelse til det underliggende væv. Blærerne ved pemphigus skyldes direkte påvirkning af autoantistofferne, mens autoantistofferne i pemphigoid udløser betændelse ved at aktivere komplement. De specifikke proteiner, som disse autoantistoffer retter sig mod, er blevet identificeret: desmogleiner i pemphigus (som er involveret i celleadhæsion) og proteiner i hemidesmosomer i pemphigoid (som forankrer celler til det underliggende lag).
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 er den mest almindelige autoimmune bulløse sygdom, der typisk rammer ældre voksne. Stigningen i antallet af tilfælde i de seneste årtier er forbundet med en aldrende befolkning, narkotikarelaterede hændelser og forbedrede diagnostiske metoder for ikke‑bulløse former af tilstanden. Det involverer en funktionsfejl i T‑celle‑responsen og produktion af autoantistoffer (IgG og IgE) rettet mod de specifikke proteiner (BP180 og BP230), hvilket resulterer i betændelse og nedbrydning af hudens støttende struktur. Symptomerne omfatter typisk blærer på hævede, kløende pletter på kroppen og på lemmerne, mens slimhinder kun sjældent er involveret. Behandlingen består primært af potente topikale og systemiske steroider, men nyere undersøgelser fremhæver fordelene og sikkerheden ved supplerende terapier såsom doxycycline, dapsone og immunosuppressanter, som har til formål at reducere steroidforbruget.
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.
