Bullous pemphigoid
Bullous pemphigoid bulla eragiten dituzten larruazaleko nahaste mota guztiak aipatzen ditu. “Pemfigoide bolutsua” larruazaleko gaixotasun autoimune bat da, gehienbat adineko pertsonengan, 60 urtekiko gorakoetan. Larruazaleko geruzen epidermiko eta dermalaren arteko espazioan babak sortzen dira pemfigoide bolutsuan.
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ReferencesPemphigus eta bullous pemphigoid autoantigorputzen ondorioz babak sortzen dituzten azaleko gaixotasunak dira. Pemphigus-en, azaleko geruzako zelulek eta muki‑mintzak elkarrekin atxikitzeko gaitasuna galtzen dute, eta pemphigoid-en, berriz, azalaren oinarrian dauden zelulek azpiko geruzarekin duten lotura galtzen dute. Pemphigus-en babak autoantigorputzak zuzenean eragiten dute, eta pemphigoid-en, berriz, autoantigorputzak hantura eragiten dute osagarria aktibatuz. Autoantigorputz horien xede diren proteina espezifikoak identifikatu dira: Pemphigus-en desmogleinak (zelulen atxikimenduan parte hartzen dituztenak) eta pemphigoid-en 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 (buloi autoimmune) gaixotasun ohikoena da, normalean helduen adineko pertsonetan eragiten du. Azken hamarkadetan izandako kasu‑gorakada biztanleria zahartzearekin, droga erabilerarekin 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 egituraren hantura eta haustura eragiten du. Sintomak normalean gorputzean eta gorputz‑adarretan azkura altxatutako orbanetan agertzen dira, muki‑mintzen inplikazio arraroarekin. Tratamendua esteroide topiko eta sistemiko indartsuetan oinarritzen da, batez ere, eta azken ikerketek doxycycline, dapsone eta 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.
