Bullous pemphigoid - Pemfigoid Bulosa
Pemfigoid Bulosa (Bullous pemphigoid) nuduhake kabeh jinis kelainan kulit sing nyebabake bulla. "Pemphigoid bullous" minangka penyakit kulit pruritik otoimun sing luwih disenengi ing wong tuwa, umure luwih saka 60. Pembentukan blisters ing spasi antarane lapisan kulit epidermis lan dermal diamati ing pemfigoid bullous.
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References
Pemphigus lan bullous pemphigoid minangka penyakit kulit ing ngendi lepuh dibentuk amarga autoantibodi. Ing pemphigus , sel ing lapisan kulit njaba lan membran mukosa kelangan kemampuan kanggo tetep bebarengan, nalika ing pemphigoid , sel ing dasar kulit kelangan sambungan karo lapisan dhasar. Lepuh pemphigus disebabake langsung dening autoantibodi, dene ing pemphigoid , autoantibodi nyebabake inflamasi kanthi ngaktifake komplemen. Protein spesifik sing ditargetake dening autoantibodi iki wis diidentifikasi: desmogleins ing pemphigus (sing melu adhesi sel) lan protein ing hemidesmosomes ing pemphigoid (kang anchor sel menyang lapisan dhasar) .
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 minangka penyakit bulosa otoimun sing paling umum, biasane mengaruhi wong tuwa. Peningkatan kasus sajrone dekade pungkasan iki ana gandhengane karo populasi sing tuwa, kedadeyan sing ana gandhengane karo obat-obatan, lan cara diagnostik sing luwih apik kanggo kondisi non-bullous. Iki kalebu malfungsi ing respon sel T lan produksi autoantibodi (IgG lan IgE) sing ngarahake protein spesifik (BP180 lan BP230) , nyebabake inflamasi lan rusak struktur pendukung kulit. Gejala biasane kalebu blistering ing wungu, gatel patch ing awak lan perangan awak, karo keterlibatan langka saka membran mucous. Perawatan utamané gumantung ing steroid topikal lan sistemik kuat, karo pasinaon anyar nyorot keuntungan lan safety saka terapi tambahan (doxycycline, dapsone, immunosuppressants) , ngarahke ing ngurangi nggunakake steroid.
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.
