Bullous pemphigoid - Bulusli Pemfigoid
Bulusli Pemfigoid (Bullous pemphigoid) buqalarni keltirib chiqaradigan barcha turdagi teri kasalliklarini anglatadi. "Bullöz pemfigoid" - bu 60 yoshdan oshgan keksa odamlarda terining otoimmun qichishishi kasalligi. Bulloz pemfigoidda epidermal va teri qatlamlari orasidagi bo'shliqda pufakchalar paydo bo'lishi kuzatiladi.
☆ Germaniyaning 2022 yilgi Stiftung Warentest natijalariga ko'ra, iste'molchilarning ModelDermdan qoniqish darajasi pullik teletibbiyot maslahatlariga qaraganda bir oz pastroq bo'lgan.
Butun tanaga ta'sir qilishi mumkin bo'lgan pufakchalar bilan qoplangan oyoqlari ko'rsatilgan fotosurat.
Boshlang'ich belgilari ba'zida ürtiker shaklida bo'ladi.
References
Pemphigus va bullous pemphigoid teri kasalliklari bo'lib, unda otoantikorlar tufayli pufakchalar paydo bo'ladi. Pemphigus da tashqi teri qavati va shilliq qavatdagi hujayralar bir-biriga yopishib qolish qobiliyatini yo'qotsa, pemphigoid da teri tagidagi hujayralar pastki qatlam bilan aloqasini yo'qotadi. Pemphigus pufakchalari to'g'ridan-to'g'ri otoantikorlar tomonidan yuzaga keladi, pemphigoid da autoantikorlar komplementni faollashtirish orqali yallig'lanishni qo'zg'atadi. Ushbu autoantikorlar tomonidan maqsad qilingan o'ziga xos oqsillar aniqlangan: pemphigus dagi desmogleinlar (hujayra yopishishida ishtirok etadi) va pemphigoid dagi gemidesmosomalardagi oqsillar (hujayralarni pastki qatlamga bog'lab turadi) .
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 eng keng tarqalgan otoimmun bullyoz kasalligi bo'lib, odatda keksa odamlarga ta'sir qiladi. So'nggi o'n yilliklarda bu holatlarning ko'payishi populyatsiyaning qarishi, giyohvand moddalar bilan bog'liq hodisalar va kasallikning bullyoz bo'lmagan shakllarini tashxislash usullarining takomillashgani bilan bog'liq. Bu T hujayralari javobidagi nosozlik va o'ziga xos oqsillarga (BP180 va BP230) qaratilgan otoantikorlarning (IgG va IgE) ishlab chiqarilishini o'z ichiga oladi, bu yallig'lanish va terining qo'llab-quvvatlovchi tuzilishining buzilishiga olib keladi. Semptomlar odatda tanada va oyoq-qo'llarda ko'tarilgan, qichiydigan pufakchalar, shilliq pardalarning kamdan-kam hollarda ishtirok etishini o'z ichiga oladi. Davolash birinchi navbatda kuchli topikal va tizimli steroidlarga tayanadi, so'nggi tadqiqotlar steroidlardan foydalanishni kamaytirishga qaratilgan doxycycline, dapsone, and immunosuppressants kabi qo'shimcha terapiyaning afzalliklari va xavfsizligini ta'kidlaydi.
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.
