Bullous pemphigoid - Pemfigoid Bulosa
Pemfigoid Bulosa (Bullous pemphigoid) nujul kana sagala jinis gangguan kulit anu nyababkeun bula. "Bullous pemphigoid" nyaéta kasakit kulit pruritic otoimun preferentially di urang heubeul, yuswa leuwih 60. Formasi lepuh dina spasi antara lapisan kulit épidermis jeung dermal dititénan dina pemphigoid bullous.
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References
Pemphigus jeung bullous pemphigoid nyaéta kasakit kulit dimana lepuh ngabentuk alatan autoantibodi. Dina pemphigus , sél dina lapisan kulit luar jeung mémbran mukosa leungit kamampuhna pikeun lengket babarengan, sedengkeun dina pemphigoid , sél dina dasar kulit leungit sambungan maranéhanana 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) sareng 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 nyaeta kasakit bullous otoimun paling umum, ilaharna mangaruhan sawawa heubeul. Paningkatan kasus dina sababaraha dasawarsa ayeuna aya hubunganana sareng populasi sepuh, insiden anu aya hubunganana sareng narkoba, sareng metode diagnostik anu ningkat pikeun bentuk non-bulous tina kaayaan éta. Éta ngalibatkeun gangguan dina réspon sél T sareng produksi autoantibodi (IgG sareng IgE) nargétkeun protéin khusus (BP180 sareng BP230) , nyababkeun peradangan sareng ngarecahna struktur anu ngadukung kulit. Gejala biasana ngawengku blistering on diangkat, patches itchy dina awak jeung anggota awak, kalawan involvement langka tina mémbran mukosa. Perlakuan utamana ngandelkeun stéroid topical jeung sistemik potent, kalawan studi panganyarna panyorot mangpaat tur kasalametan terapi tambahan (doxycycline, dapsone, immunosuppressants) , aimed dina ngurangan pamakéan stéroid.
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.
