Bullous pemphigoid
Bullous pemphigoid ቡላዎችን የሚያነሳሱ ሁሉንም ዓይነት የቆዳ በሽታዎችን ያመለክታል። "Bullous pemphigoid" - ኦቶኢሙን (autoimmune) የቆዳ በሽታ ነው በዕድሜ የገፉ ሰዎች, ዕድሜያቸው ከ 60 ዓመት በላይ. በ epidermis እና dermal የቆዳ ንብርብሮች መካከል ያለውን ክፍተት ውስጥ አረፋ ምስረታ bullous pemphigoid ውስጥ ይታያል.
☆ እ.ኤ.አ. በ 2022 ከጀርመን የስቲፍቱንግ ዋረንቴስት ውጤቶች ፣ በሞዴልደርም የተገልጋዮች እርካታ ከሚከፈልባቸው የቴሌሜዲኬን ምክሮች በትንሹ ያነሰ ነበር።
እግሮቹን ብቅ ባሉ አረፋዎች ተሸፍነው የሚያሳይ ፎቶ፣ ይህም መላ ሰውነትን ሊጎዳ ይችላል።
የመጀመሪያ ምልክቶች አንዳንድ ጊዜ በቀፎ መልክ ይታያሉ።
relevance score : -100.0%
ReferencesPemphigus እና bullous pemphigoid በራስ-አንቲቦዲዎች ምክንያት አረፋዎች የሚፈጠሩ የቆዳ በሽታዎች ናቸው። በ pemphigus በውጫዊው የቆዳ ሽፋን ውስጥ ያሉ ሴሎች እና የ mucous membranes አንድ ላይ ተጣብቀው የመቆየት ችሎታቸውን ያጣሉ, በ pemphigoid ደግሞ በቆዳው ስር ያሉ ሴሎች ከታችኛው ሽፋን ጋር ያላቸውን ግንኙነት ያጣሉ. የ pemphigus አረፋዎች በቀጥታ በአውቶአንቲቦዲዎች የሚከሰቱ ሲሆን በ pemphigoid ውስጥ ግን ራስ-አንቲቦዲዎች ማሟያ (complement) በማንቃት እብጠትን ያስከትላሉ። በእነዚህ ራስ-አንቲቦዲዎች የታለሙት ልዩ ፕሮቲኖች ተለይተዋል፡- ዴስሞግሊንስ በ pemphigus (በሴሎች መጣበቅ ውስጥ የሚሳተፉ) እና በ pemphigoid (ሴሎች ላይ ወደታችኛው ሽፋን የሚያርቁ) hemidesmosomes ውስጥ ያሉ ፕሮቲኖች።
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 በጣም የተለመደ ራስን የመከላከል ቡልስ (bullous) ፔምፊጎይድ (pemphigoid) በሽታ፣በተለምዶ አዛውንቶችን የሚያጠቃ ነው። ከቅርብ አሥርተ ዓመታት ወዲህ ያለው የጉዳይ መጨመር ከእርጅና ጋር የተቆራኘ ነው፣ ከመድኃኒት ጋር በተያያዙ ጉዳዮች፣ እና ለበሽታ ላልሆኑ ዓይነቶች የተሻሻሉ የምርመራ ዘዴዎች። በቲ-ሴል (T cell) ምላሽ ላይ ብልሽትን ያካትታል እና የተወሰኑ ፕሮቲኖችን (BP180 እና BP230) ላይ ያነጣጠሩ አውቶአንቲቦዲዎች (IgG እና IgE) መፈጠርን ያካትታል ይህም የቆዳውን የድጋፍ መዋቅር መበላሸትን ያስከትላል። ምልክቶቹ ብዙውን ጊዜ ተገባች ብልሽቶች በኦርቲካሪያል ፕላክ ላይ በአካል እና በእግር ላይ ይታያሉ። ሕክምናው በዋነኝነት በኃይለኛ የቦታዊ እና የሲስተም ስተሮይዶች (steroids) ላይ የተመሰረተ ነው፣ በቅርብ ጊዜ የተደረጉ ጥናቶች የስተሮይድ (steroid) አጠቃቀምን ለመቀነስ የታለሙ ተጨማሪ ሕክምናዎች (doxycycline, dapsone, immunosuppressants) ጥቅሞችን እና ደህንነትን አጉልተው ያሳያሉ።
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.
