Bullous pemphigoid - Pemfigoid Bulluż
Pemfigoid Bulluż (Bullous pemphigoid) tirreferi għal kull tip ta' disturbi fil-ġilda li jinduċu bullaes. "Pemphigoid bullous" hija marda tal-ġilda pruritika awtoimmuni preferenzjali f'nies anzjani, ta 'età 'l fuq minn 60. Il-formazzjoni ta' infafet fl-ispazju bejn is-saffi tal-ġilda epidermali u dermali hija osservata f'penfigoid bullous.
☆ Fir-riżultati ta' Stiftung Warentest tal-2022 mill-Ġermanja, is-sodisfazzjon tal-konsumatur b'ModelDerm kien biss ftit inqas milli b'konsultazzjonijiet bit-telemediċina mħallsa.
Ritratt li juri s-saqajn miksija b'folji popped, li jistgħu jaffettwaw il-ġisem kollu.
Is-sintomi inizjali kultant huma fil-forma ta 'ħorriqija.
References
Pemphigus u bullous pemphigoid huma mard tal-ġilda fejn jiffurmaw infafet minħabba awtoantikorpi. F’ pemphigus , iċ-ċelloli fis-saff ta’ barra tal-ġilda u l-membrani mukużi jitilfu l-kapaċità tagħhom li jeħlu flimkien, filwaqt li f’ pemphigoid , iċ-ċelloli fil-bażi tal-ġilda jitilfu l-konnessjoni tagħhom mas-saff ta’ taħt. Il-folji ta' pemphigus huma kkawżati direttament mill-awtoantikorpi, filwaqt li f' pemphigoid , l-awtoantikorpi jikkawżaw infjammazzjoni billi jattivaw il-kompliment. Il-proteini speċifiċi mmirati minn dawn l-awtoantikorpi ġew identifikati: desmogleins f' pemphigus (li huma involuti fl-adeżjoni taċ-ċelluli) u proteini f'emidemosomi f' pemphigoid (li jankraw iċ-ċelloli mas-saff ta' taħt) .
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 hija l-aktar marda bullous awtoimmuni komuni, li tipikament taffettwa lill-adulti anzjani. Iż-żieda fil-każijiet matul l-aħħar deċennji hija marbuta ma 'popolazzjonijiet li qed jixjieħu, inċidenti relatati mad-droga, u metodi dijanjostiċi mtejba għal forom mhux bullous tal-kundizzjoni. Tinvolvi ħsara fir-rispons taċ-ċelluli T u l-produzzjoni ta 'awtoantikorpi (IgG u IgE) li jimmiraw proteini speċifiċi (BP180 u BP230) , li jirriżultaw f'infjammazzjoni u tkissir tal-istruttura ta' appoġġ tal-ġilda. Is-sintomi normalment jinkludu nfafet fuq irqajja mgħollija u ħakk fuq il-ġisem u r-riġlejn, b'involviment rari tal-membrani mukużi. It-trattament tiddependi primarjament fuq sterojdi topiċi u sistemiċi qawwija, bi studji reċenti li jenfasizzaw il-benefiċċji u s-sigurtà ta 'terapiji addizzjonali (doxycycline, dapsone, immunosuppressants) , immirati biex inaqqsu l-użu ta' sterojdi.
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.
