Bullous pemphigoid - Penfigoide Bulloso
Penfigoide Bulloso (Bullous pemphigoid) refírese a todo tipo de trastornos da pel que inducen bullas. O "penfigoide ampollar" é unha enfermidade da pel pruriginosa autoinmune preferentemente en persoas maiores, maiores de 60 anos. A formación de burbullas no espazo entre as capas da pel epidérmica e dérmica obsérvase no penfigoide ampollar.
☆ Nos resultados de Stiftung Warentest de Alemaña de 2022, a satisfacción dos consumidores con ModelDerm foi só lixeiramente inferior á das consultas de telemedicina pagas.
Unha foto que mostra as pernas cubertas de burbullas, que poden afectar a todo o corpo.
Os síntomas iniciais son ás veces en forma de urticaria.
References
Pemphigus e bullous pemphigoid son enfermidades da pel nas que se forman burbullas debido a autoanticorpos. En pemphigus , as células da capa exterior da pel e as membranas mucosas perden a súa capacidade de unirse, mentres que en pemphigoid , as células da base da pel perden a súa conexión coa capa subxacente. As burbullas de pemphigus son causadas directamente polos autoanticorpos, mentres que en pemphigoid , os autoanticorpos desencadean a inflamación activando o complemento. Identificáronse as proteínas específicas dirixidas a estes autoanticorpos: desmogleínas en pemphigus (que están implicadas na adhesión celular) e proteínas nos hemidesmosomas en pemphigoid (que ancoran as células á capa subxacente) .
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 é a enfermidade bullosa autoinmune máis común, que adoita afectar aos adultos maiores. O aumento dos casos nas últimas décadas está relacionado co envellecemento da poboación, os incidentes relacionados coas drogas e a mellora dos métodos de diagnóstico para as formas non bullosas da enfermidade. Implica un mal funcionamento na resposta dos linfocitos T e na produción de autoanticorpos (IgG e IgE) dirixidos a proteínas específicas (BP180 e BP230) , o que provoca inflamación e ruptura da estrutura de soporte da pel. Os síntomas adoitan incluír ampollas en parches elevados e pruriginosos no corpo e nas extremidades, con afectación rara das membranas mucosas. O tratamento depende principalmente de potentes esteroides tópicos e sistémicos, con estudos recentes que destacan os beneficios e a seguridade de terapias adicionais (doxycycline, dapsone, immunosuppressants) , dirixidas a reducir o uso de esteroides.
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.
