Kussini Jacqueline, Kaisers Tabea, Sequeira Santos Antonio Manuel, Eming Rüdiger, Didona Dario
Klinik für Dermatologie und Allergologie, Philipps-Universität Marburg, Baldingerstr., 35043, Marburg, Deutschland.
Klinik III Dermatologie, Venerologie & Allergologie, Bundeswehrzentralkrankenhaus Koblenz, Koblenz, Deutschland.
Dermatologie (Heidelb). 2022 Dec;74(12):915-926. doi: 10.1007/s00105-023-05246-y. Epub 2023 Oct 30.
Pemphigus diseases are a group of organ-specific autoimmune diseases which are characterised by the production of autoantibodies against intra-epidermal adhesion molecules and structural proteins of skin and mucosae. Depending on the entity, patients develop blisters and erosions on the skin and/or mucosae. According to the AWMF S2k guidelines for diagnosis and therapy of pemphigus diseases, a systemic therapy is recommended. Initially, high-dose, oral corticosteroids in combination with immunosuppressive drugs as corticosteroid-sparing agent, usually azathioprine or mycophenolate mofetil, can be used. Furthermore, rituximab, a monoclonal antibody directed against CD20 on B cells, was recently approved for pemphigus vulgaris and moderate or severe pemphigus foliaceus.
天疱疮疾病是一组器官特异性自身免疫性疾病,其特征是针对表皮内黏附分子以及皮肤和黏膜的结构蛋白产生自身抗体。根据具体类型,患者会在皮肤和/或黏膜上出现水疱和糜烂。根据德国医学专业协会(AWMF)天疱疮疾病诊断和治疗的S2k指南,建议进行全身治疗。最初,可以使用高剂量口服糖皮质激素联合免疫抑制药物作为糖皮质激素节约剂,通常是硫唑嘌呤或霉酚酸酯。此外,利妥昔单抗,一种针对B细胞上CD20的单克隆抗体,最近已被批准用于寻常型天疱疮和中度或重度落叶型天疱疮。