Powell A M, Albert S, Oyama N, Sakuma-Oyama Y, Bhogal B, Black M M
Immunofluorescence Department, St John's Institute of Dermatology, St Thomas' Hospital, Lambeth Palace Road, London SE1 7EH, UK.
J Eur Acad Dermatol Venereol. 2004 May;18(3):360-4. doi: 10.1111/j.1468-3083.2004.00917.x.
We report a patient with chronic lymphocytic leukaemia who developed paraneoplastic pemphigus (PNP) soon after the initiation of fludarabine therapy. He presented with severe oral and cutaneous erosions. Initially, he had high titres of circulating autoantibodies as detected by indirect immunofluorescence (IF) on multiple epithelial substrates (normal human skin, monkey oesophagus, and rat bladder) and by desmoglein 1 and 3 enzyme-linked immunosorbent assays (ELISAs). His oral erosions have subsequently progressed into unusual hyperplastic papillomatous lesions affecting the inner aspect of lips and buccal mucosae, histologically consistent with pemphigus vegetans. Desmoglein 1 antibodies and IF on rat bladder substrate have become negative after 18 months of therapy. Several agents had been initiated to bring the disease under control originally, but a partial remission was achieved and maintained with mycophenolate mofetil and low-dose prednisolone.
我们报告了一名慢性淋巴细胞白血病患者，在接受氟达拉滨治疗后不久发生了副肿瘤性天疱疮（PNP）。他出现了严重的口腔和皮肤糜烂。最初，通过在多种上皮底物（正常人皮肤、猴食管和大鼠膀胱）上进行间接免疫荧光（IF）以及桥粒芯糖蛋白1和3酶联免疫吸附测定（ELISA）检测到他有高滴度的循环自身抗体。随后，他的口腔糜烂进展为影响嘴唇内侧和颊黏膜的异常增生性乳头状病变，组织学上与增殖性天疱疮一致。治疗18个月后，桥粒芯糖蛋白1抗体和大鼠膀胱底物上的IF检测结果变为阴性。最初曾使用多种药物来控制病情，但使用霉酚酸酯和小剂量泼尼松龙后实现并维持了部分缓解。
