Department of Dermatology, University of Cincinnati, College of Medicine, 231 Albert Sabin Way, P.O. Box 670592, Cincinnati, OH 45267-0592, USA.
Drugs Aging. 2010 Jan 1;27(1):1-19. doi: 10.2165/11318600-000000000-00000.
Elderly individuals are susceptible to autoimmune bullous dermatoses (ABDs), which may be associated with high morbidity and mortality. ABDs result from an autoimmune response to components of the basement membrane zone at the dermal-epidermal junction or desmosomes. Bullous pemphigoid results from autoimmunity to hemidesmosomal proteins present in the basement membrane of stratified squamous epithelia. Patients present with tense blisters in flexural areas of the skin. Mild disease may be treated with potent topical corticosteroids, while extensive disease usually requires systemic corticosteroids or systemic immunosuppressive agents such as azathioprine. Mucosal pemphigoid affects one or more mucous membranes that are lined by stratified squamous epithelia. The two most commonly involved sites are the eye and the oral cavity. Lesions frequently result in scar formation that may cause blindness. Patients with severe disease or ocular involvement require aggressive therapy with corticosteroids and cyclophosphamide. Epidermolysis bullosa acquisita results from autoimmunity to type VII collagen in the anchoring fibrils of the basement membrane. Lesions may either arise on an inflammatory base or be non-inflammatory and result primarily from trauma. Treatment options include corticosteroids, dapsone, ciclosporin, methotrexate and plasmapheresis/immunoapheresis. Paraneoplastic pemphigus results from autoimmunity to multiple desmosomal antigens. The disorder is associated with neoplasms, especially leukaemia, lymphoma and thymoma. Patients present with stomatitis and polymorphous skin eruption. The disease may respond to successful treatment of the underlying neoplasm or may require immunosuppressive therapy.
老年人易患自身免疫性大疱性皮肤病 (ABDs)，其发病率和死亡率较高。ABDs 是由于自身免疫反应导致真皮-表皮交界处基底膜带或桥粒的成分受损引起的。大疱性类天疱疮是由桥粒蛋白自身免疫引起的，这些蛋白存在于复层鳞状上皮的基底膜中。患者表现为皮肤屈侧紧张性水疱。轻度疾病可采用强效局部皮质类固醇治疗，而广泛疾病通常需要全身皮质类固醇或全身免疫抑制剂，如硫唑嘌呤。黏膜类天疱疮影响一个或多个由复层鳞状上皮衬里的黏膜。最常受累的两个部位是眼睛和口腔。病变常导致瘢痕形成，可能导致失明。严重疾病或眼部受累的患者需要使用皮质类固醇和环磷酰胺进行积极治疗。获得性大疱性表皮松解症是由基底膜锚定纤维中的 VII 型胶原自身免疫引起的。病变可能在炎症基础上发生，也可能是非炎症性的，主要由创伤引起。治疗选择包括皮质类固醇、氨苯砜、环孢素、甲氨蝶呤和血浆置换/免疫吸附。副肿瘤性天疱疮是由多种桥粒抗原自身免疫引起的。该疾病与肿瘤有关，特别是白血病、淋巴瘤和胸腺瘤。患者表现为口炎和多形性皮肤疹。该疾病可能对潜在肿瘤的成功治疗有反应，也可能需要免疫抑制治疗。
