Wallet-Faber N, Franck N, Batteux F, Mateus C, Gilbert D, Carlotti A, Avril M F, Dupin N
Service de Dermatologie et Vénéréologie, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Paris, France.
Dermatology. 2007;215(3):252-5. doi: 10.1159/000106585.
Epidermolysis bullosa acquisita is a rare autoimmune subepidermal blistering disease, often resisting current treatments, especially systemic corticosteroids. We report a patient having a bullous pemphigoid who relapsed with clinical and immunological features of inflammatory epidermolysis bullosa acquisita. An anti-CD20 monoclonal antibody (rituximab) was proposed because of resistance to high-dose steroids and other immunosuppressive agents. The disease dramatically improved within a few weeks following rituximab infusion allowing the decrease in steroid therapy. Our case illustrates also the possible evolution from bullous pemphigoid to epidermolysis bullosa acquisita that should be suspected when clinical atypia occurs or in case of corticosteroid resistance.
获得性大疱性表皮松解症是一种罕见的自身免疫性表皮下大疱性疾病，常常对当前治疗方法，尤其是全身性皮质类固醇治疗产生抵抗。我们报告了一例患有大疱性类天疱疮的患者，其复发时具有炎性获得性大疱性表皮松解症的临床和免疫学特征。由于对高剂量类固醇和其他免疫抑制剂耐药，故使用了抗CD20单克隆抗体（利妥昔单抗）。在输注利妥昔单抗后的几周内，病情显著改善，从而得以减少类固醇治疗。我们的病例还说明了大疱性类天疱疮可能演变为获得性大疱性表皮松解症，当出现临床异型或皮质类固醇耐药时应怀疑这种情况。
