Bullous pemphigoid - Bulløs Pemphigoid
Bulløs pemfigoid (Bullous pemphigoid) refererer til alle slags hudlidelser, der fremkalder bullaer. "Bulløs pemfigoid (Bullous pemphigoid)" er en autoimmun kløende hudsygdom, fortrinsvis hos ældre mennesker over 60 år. Dannelsen af blærer i mellemrummet mellem de epidermale og dermale hudlag observeres hos bulløs pemfigoid (Bullous pemphigoid).
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De første symptomer er nogle gange i form af nældefeber.
relevance score : -100.0%
ReferencesPemfigus (pemphigus) og Bulløs pemfigoid (bullous pemphigoid) er hudsygdomme, hvor der dannes blærer på grund af autoantistoffer. I pemfigus mister celler i det ydre hudlag og slimhinder deres evne til at klæbe sammen, mens i bulløs pemfigoid mister celler i bunden af huden deres forbindelse til det underliggende lag. Blærerne af pemfigus er forårsaget direkte af autoantistofferne, mens i bulløs pemfigoid udløser autoantistofferne betændelse ved at aktivere komplement. De specifikke proteiner målrettet af disse autoantistoffer er blevet identificeret: desmogleiner i pemfigus (som er involveret i celleadhæsion) og proteiner i hemidesmosomer i bulløs pemfigoid (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).
Bulløs pemfigoid (Bullous pemphigoid) er den mest almindelige autoimmune bulløse sygdom, der typisk rammer ældre voksne. Stigningen i tilfælde i de seneste årtier er forbundet med aldrende befolkninger, medikamentinducerede tilfælde (drug-induced cases) og forbedrede diagnostiske metoder for ikke‑bulløse former for tilstanden. Det involverer en funktionsfejl i T‑cellerespons og produktion af autoantistoffer (IgG og IgE) rettet mod specifikke proteiner (BP180 og BP230), hvilket resulterer i betændelse og nedbrydning af hudens støttende struktur. Symptomerne omfatter sædvanligvis blærer på hævede, kløende pletter på kroppen og lemmerne, med sjælden involvering af slimhinder. Behandlingen er primært afhængig af potente topikale og systemiske steroider, med nyere undersøgelser, der fremhæver fordelene og sikkerheden ved yderligere terapier som doxycycline, dapsone og immunosuppressiva (immunosuppressants), der sigter mod at reducere brugen af steroider.
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.
