Bullous pemphigoid
Bullous pemphigoid bullaes eragiten duten larruazaleko nahaste mota guztiak aipatzen ditu. "Pemfigoide bolutsua" larruazaleko gaixotasun autoimmune bat da, lehentasunez adineko pertsonengan, 60 urtetik gorakoak. Larruazaleko geruzen epidermiko eta dermalaren arteko espazioan babak sortzen dira pemfigoide bolosoan.
☆ Alemaniako Stiftung Warentest-en 2022ko emaitzetan, ModelDerm-ekin kontsumitzaileen gogobetetasuna apur bat txikiagoa izan zen ordaindutako telemedikuntzako kontsultekin baino.
Gorputz osoari eragin diezaiokeen hankak babak estalita erakusten dituen argazkia.
Hasierako sintomak batzuetan erlauntza formakoak dira.
References
Pemphigus eta bullous pemphigoid autoantigorputzen ondorioz babak sortzen diren azaleko gaixotasunak dira. Pemphigus -n, kanpoko azaleko geruzako zelulek eta muki-mintzek elkarrekin atxikitzeko gaitasuna galtzen dute, eta pemphigoid -n, berriz, azalaren oinarrian dauden zelulek azpiko geruzarekin duten lotura galtzen dute. Pemphigus -ren babak autoantigorputzak zuzenean eragiten ditu, eta pemphigoid -n, berriz, autoantigorputzak hantura eragiten du osagarria aktibatuz. Autoantigorputz horien xede diren proteina espezifikoak identifikatu dira: pemphigus -n desmogleinak (zelulen atxikimenduan parte hartzen dutenak) eta pemphigoid -ko hemidesmosometako proteinak (zelulak azpiko geruzara ainguratzen dituztenak) .
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 gaixotasun buloi autoimmune ohikoena da, normalean adineko helduei eragiten diena. Azken hamarkadetan izandako kasuen gorakada biztanleria zahartzearekin, drogekin lotutako gertakariekin eta gaixotasunaren forma ez-bulosoen diagnostiko-metodo hobeekin lotuta dago. T zelulen erantzunean funtzionamendu okerra eta proteina espezifikoak (BP180 eta BP230) bideratzen dituzten autoantigorputzak (IgG eta IgE) ekoiztea dakar, eta larruazaleko egitura euskarriaren hantura eta haustura eragiten du. Sintomak normalean gorputzean eta gorputz-adarretan azkura altxatutako orbanetan babak sartzen dira, muki-mintzen inplikazio arraroarekin. Tratamendua esteroide topiko eta sistemiko indartsuetan oinarritzen da batez ere, eta azken ikerketek doxycycline, dapsone, and immunosuppressants bezalako terapia osagarrien onurak eta segurtasuna nabarmendu dituzte, esteroideen erabilera murrizteko helburuarekin.
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.
