Bullous pemphigoid - Penfigoide Bulloso
Penfigoide Bulloso (Bullous pemphigoid) si riferisce à tutti i tipi di disordini di a pelle chì inducenu bullaes. "Bullous pemphigoid" hè una malatia autoimmune pruriginosa di a pelle preferibilmente in e persone anziane, in età di più di 60. A furmazione di blisters in u spaziu trà i strati di a pelle epidermica è dermica hè osservata in pemphigoid bullous.
☆ In i risultati Stiftung Warentest 2022 da Germania, a satisfaczione di i cunsumatori cù ModelDerm era solu ligeramente più bassu chè cù cunsultazioni di telemedicina pagate.
Una foto chì mostra i gammi cuparti di vesciche, chì ponu influenzà tuttu u corpu.
I sintomi iniziali sò qualchì volta in forma di bugni.
References
Pemphigus è bullous pemphigoid sò e malatie di a pelle induve si formanu vesciche per via di l'autoanticorpi. In pemphigus , e cellule in a capa esterna di a pelle è a mucosa perde a so capacità di aderisce, mentre chì in pemphigoid , e cellule à a basa di a pelle perde a so cunnessione cù a capa sottostante. I blisters di pemphigus sò causati direttamente da l'autoantibodies, mentri in pemphigoid , l'autoantibodies provocanu l'infiammazione attivendu u cumplementu. E proteine specifiche mirate da questi autoanticorpi sò state identificate: desmogleins in pemphigus (chì sò implicati in l'aderenza cellulare) è proteine in hemidesmosomi in pemphigoid (chì ancoranu e cellule à a capa sottostante) .
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 hè a malatia bullosa autoimmune più cumuna, chì affetta tipicamente l'adulti anziani. L'aumentu di i casi in l'ultimi decennii hè ligatu à l'anziane di pupulazioni, incidenti ligati à a droga, è metudi di diagnostichi perfezionati per e forme non-bullose di a cundizione. Implica un malfunzionamentu in a risposta di e cellule T è a produzzione d'autoanticorpi (IgG è IgE) destinati à proteine specifiche (BP180 è BP230) , chì risultanu in inflammazioni è rotture di a struttura di supportu di a pelle. I sintomi di solitu includenu vesciche nantu à pezze rialzate, pruriginate nantu à u corpu è i membri, cù rara implicazione di e mucosa. U trattamentu si basa principalmente in steroidi topichi è sistemichi potenti, cù studii recenti chì mette in risaltu i benefici è a sicurità di terapie supplementari (doxycycline, dapsone, immunosuppressants) , destinate à riduce l'usu di steroidi.
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.
