Bullous pemphigoid
Bullous pemphigoid inarejelea aina zote za matatizo ya ngozi ambayo husababisha bulla. "Bullous pemphigoid" ni ugonjwa wa ngozi ya pruritic ya autoimmune kwa upendeleo kwa watu wazee, wenye umri wa zaidi ya miaka 60. Uundaji wa malengelenge katika nafasi kati ya tabaka za ngozi na ngozi huzingatiwa katika pemphigoid ya bullous.
☆ Katika matokeo ya 2022 ya Stiftung Warentest kutoka Ujerumani, kuridhika kwa watumiaji na ModelDerm kulikuwa chini kidogo kuliko na mashauriano ya matibabu ya simu yanayolipishwa.
Picha inayoonyesha miguu iliyofunikwa na malengelenge, ambayo inaweza kuathiri mwili mzima.
Dalili za awali wakati mwingine huwa katika mfumo wa mizinga.
References
Pemphigus na bullous pemphigoid ni magonjwa ya ngozi ambapo malengelenge hutokea kutokana na kingamwili. Katika pemphigus , seli kwenye safu ya ngozi ya nje na utando wa mucous hupoteza uwezo wao wa kushikamana, huku kwenye pemphigoid , seli zilizo chini ya ngozi hupoteza muunganisho wao kwenye safu ya chini. Malengelenge ya pemphigus husababishwa moja kwa moja na kingamwili, ilhali katika pemphigoid , kingamwili huchochea kuvimba kwa kuwezesha kijalizo. Protini mahususi zinazolengwa na kingamwili hizi zimetambuliwa: desmogleins katika pemphigus (ambazo huhusika katika kushikamana kwa seli) na protini katika hemidesmosomes katika pemphigoid (ambazo hushikilia seli kwenye safu ya chini) .
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 ndio ugonjwa unaojulikana zaidi wa ng'ombe wa kingamwili, ambao kwa kawaida huwaathiri watu wazima. Kuongezeka kwa kesi katika miongo ya hivi majuzi kunahusishwa na idadi ya watu wanaozeeka, matukio yanayohusiana na dawa za kulevya, na njia bora za utambuzi kwa aina zisizo za dhuluma za hali hiyo. Inahusisha utendakazi mbaya katika mwitikio wa seli T na utengenezaji wa kingamwili (IgG na IgE) inayolenga protini maalum (BP180 na BP230) , na kusababisha kuvimba na kuvunjika kwa muundo wa ngozi. Dalili kawaida hujumuisha malengelenge kwenye mabaka yaliyoinuliwa, kuwasha kwenye mwili na miguu na mikono, na utando wa mucous uhusika kwa nadra. Matibabu kimsingi hutegemea steroidi zenye nguvu na za kimfumo, huku tafiti za hivi majuzi zikiangazia manufaa na usalama wa matibabu ya ziada (doxycycline, dapsone, immunosuppressants) , yanayolenga kupunguza matumizi ya 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.
