Scully Crispian, Mignogna Michele
University College London, Eastman Dental Institute, London, UK.
Br J Oral Maxillofac Surg. 2008 Jun;46(4):272-7. doi: 10.1016/j.bjoms.2007.07.205. Epub 2007 Sep 17.
Pemphigus defines a group of rare mucocutaneous autoimmune diseases of which pemphigus vulgaris (PV) is the most common. The aetiology and pathogenesis of PV are not completely clear, but there is a fairly strong genetic background: ethnic groups such as Ashkenazi Jews and people of Mediterranean and Indian origin are particularly susceptible and there is a link to HLA class II alleles. The initiating event in PV is not clear, but circulating IgG autoantibodies develop, directed particularly against the intercellular cadherin desmoglein 3 (Dsg3) in desmosomes of stratified squamous epithelium. Oral lesions often herald the disease and are initially vesiculobullous, but they rupture readily to leave ulcers. Involvement of other mucosa and skin is almost inevitable and PV is potentially life threatening. The diagnosis is confirmed by biopsy with histological examination and immunostaining. Management is largely by systemic immunosuppression with corticosteroids, usually azathioprine or other agents, but newer treatments with potentially fewer adverse effects look promising.
天疱疮是一组罕见的黏膜皮肤自身免疫性疾病，其中寻常型天疱疮（PV）最为常见。PV的病因和发病机制尚不完全清楚，但存在相当强的遗传背景：德系犹太人以及地中海和印度裔等族群尤其易感，且与人类白细胞抗原（HLA）II类等位基因有关。PV的起始事件尚不清楚，但会产生循环IgG自身抗体，这些抗体尤其针对复层鳞状上皮桥粒中的细胞间钙黏蛋白桥粒芯糖蛋白3（Dsg3）。口腔病变常为该病的先兆，起初为水疱大疱性，但很容易破裂形成溃疡。其他黏膜和皮肤受累几乎不可避免，PV可能危及生命。通过活检进行组织学检查和免疫染色可确诊。治疗主要是使用皮质类固醇进行全身免疫抑制，通常联合硫唑嘌呤或其他药物，但具有潜在较少不良反应的新疗法前景看好。
