Bullous pemphigoid
Bullous pemphigoid vísar til alls kyns húðsjúkdóma sem valda bulla. „Bullous pemphigoid“ er sjálfsofnæmis kláðahúðsjúkdómur helst hjá eldra fólki, eldri en 60 ára. Myndun blaðra í bilinu á milli húðþekju- og húðlaganna kemur fram hjá bólusótt.
☆ Í niðurstöðum Stiftung Warentest 2022 frá Þýskalandi var ánægja neytenda með ModelDerm aðeins minni en með greiddum fjarlækningaráðgjöfum.
Mynd sem sýnir fætur þakta blöðrum sem geta haft áhrif á allan líkamann.
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References
Pemphigus og bullous pemphigoid eru húðsjúkdómar þar sem blöðrur myndast vegna sjálfsmótefna. Í pemphigus missa frumur í ytra húðlagi og slímhúð hæfni sinni til að festast saman en í pemphigoid missa frumur við botn húðarinnar tengingu við undirliggjandi lag. Blöðrur af pemphigus stafa beint af sjálfsmótefnunum, en í pemphigoid koma sjálfsmótefnin af stað bólgu með því að virkja komplement. Sérstök prótein sem þessi sjálfsmótefni miða á hafa verið auðkennd: desmogleins í pemphigus (sem taka þátt í frumuviðloðun) og prótein í hemidesmosomes í pemphigoid (sem festa frumur við undirliggjandi lag) .
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 er algengasti sjálfsofnæmissjúkdómurinn, sem hefur venjulega áhrif á eldra fólk. Fjölgun tilfella undanfarna áratugi tengist öldrun íbúa, lyfjatengdum atvikum og bættum greiningaraðferðum fyrir sjúkdóminn sem ekki er bullandi. Það felur í sér bilun í svörun T-frumna og framleiðslu sjálfsmótefna (IgG og IgE) sem miða að sérstökum próteinum (BP180 og BP230) , sem leiðir til bólgu og niðurbrots stuðningsbyggingar húðarinnar. Einkenni eru venjulega blöðrur á upphleyptum, kláða blettum á líkama og útlimum, með sjaldgæfum þáttum í slímhúð. Meðferð byggir fyrst og fremst á öflugum staðbundnum og almennum sterum, þar sem nýlegar rannsóknir hafa sýnt fram á ávinning og öryggi viðbótarmeðferða (doxycycline, dapsone, immunosuppressants) , sem miða að því að draga úr steranotkun.
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.
