Bullous pemphigoid - Pemfigoid Bulosa
Pemfigoid Bulosa (Bullous pemphigoid) nujul kana sagala jinis gangguan kulit anu nyababkeun bula. "Bullous pemphigoid" nyaéta kasakit kulit anu ngabalukarkeun gatel, autoimun, utamana kapanggih dina jalma nu geus kolot, yuswa leuwih ti 60 taun. Formasi lepuh dina spasi antara lapisan kulit épidermis jeung dermal dititénan dina Pemfigoid Bulosa (Bullous pemphigoid).
☆ AI Dermatology — Free Service
Dina hasil Stiftung Warentest 2022 ti Jerman, kapuasan konsumen sareng ModelDerm ngan ukur langkung handap tibatan konsultasi telemedicine anu mayar.
Dina hasil Stiftung Warentest 2022 ti Jerman, kapuasan konsumen sareng ModelDerm ngan ukur langkung handap tibatan konsultasi telemedicine anu mayar.
Poto némbongkeun suku nu ditutupan ku lepuh (blister) anu pecah, nu tiasa mangaruhan sakabéh awak.
Gejala awal kadang muncul dina bentuk hives.
relevance score : -100.0%
ReferencesPemphigus jeung bullous pemphigoid nyaéta kasakit kulit anu ditandaan ku lepuh alatan autoantibodi. Dina pemphigus, sél dina lapisan epidermis jeung mémbran mukosa leungit kamampuhna pikeun nempel silih, sedengkeun dina pemphigoid, sél dina lapisan dermis leungit sambungan ka lapisan dasar. Lepuh pemphigus disababkeun langsung ku autoantibodi, sedengkeun dina pemphigoid, autoantibodi memicu peradangan ku ngaktifkeun komplemén. Protéin spésifik anu ditargetkeun ku autoantibodi ieu parantos diidentifikasi: desmogleins dina pemphigus (anu kalibet dina adhesion sél) jeung protéin dina hemidesmosomes dina pemphigoid (anu jangkar sél kana lapisan dasar).
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 nyaéta kasakit bullous otoimun paling umum, ilaharna mangaruhan jalma déwasa anu kolot. Paningkatan kasus dina sababaraha dasawarsa ayeuna patali jeung populasi sepuh, insiden anu patali jeung panggunaan narkoba, jeung metoda diagnostik anu ningkat pikeun bentuk non‑bulous tina kaayaan éta. Éta ngalibatkeun gangguan dina réspon sél T jeung produksi autoantibodi (IgG sareng IgE) nu nargétkeun protéin khusus (BP180 sareng BP230), nu nyababkeun peradangan jeung ngarecahna struktur anu ngadukung kulit. Gejala biasana ngawengku blistering anu muncul, patch anu gatel dina awak jeung anggota awak, kalayan keterlibatan langka tina mémbran mukosa. Pangobatan utamana ngandelkeun kortikostéron topikal jeung sistemik anu kuat, kalayan studi panganyarna nu nyorot mangpaat jeung kasalametan terapi tambahan (doxycycline, dapsone, immunosuppressants), nu ditujukeun pikeun ngurangan pamakéan kortikostéron.
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.
