Pemphigus is a chronic autoimmune blistering disorder, characterized by (muco-)cutaneous erosions due to autoantibodies against desmoglein 3 and/or 1. Pemphigus induction might be associated with drugs, malignancy or radiation therapy (RT); the latter being only rarely described. A rigorous literature review revealed around 30 cases of RT-associated pemphigus, which had been primarily treated with topical and/or systemic steroids, in some cases also dapsone or few other immunosuppressive agents were given. The most common underlying cancer type was breast cancer. We here present a 63-year-old male patient, who was pre-treated with adjuvant RT for larynx carcinoma 3 months before admission. He developed extensive cutaneous, ocular, and oral erosions. Despite the clinical picture comparable to a paraneoplastic pemphigus, the diagnosis of pemphigus vulgaris of mucocutaneous type was established based on the direct immunofluorescence, showing positive cell surface IgG and discrete C3 deposits, with matching cell surface IgG pattern on monkey esophagus. Serum autoantibodies to desmoglein 1 and 3 were highly positive. No further autoantibodies were found, thus paraneoplastic pemphigus was excluded. The patient was treated with high dose prednisolone, partially given intravenously up to 2 mg/kg per day, as well as topical disinfectants and class IV steroid cream. To stabilize the disease rituximab 2 × 1,000 mg was given, leading to clinical and serological remission for up to 2 years now. We show that rituximab represents a good treatment option for the frequently treatment-refractory RT-associated pemphigus, a clinically and immunologically specific RT-induced skin disorder, resulting in long-term clinical, and serological remission.

中文翻译：

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先前患有恶性肿瘤的患者中与辐射相关的寻常性天疱疮：使用利妥昔单抗作为重要选择。

天疱疮是一种慢性自身免疫性水疱性疾病，其特征是由于抗桥粒芯蛋白3和/或1的自身抗体引起的（粘膜）皮肤糜烂。天疱疮的诱导可能与药物，恶性肿瘤或放射治疗（RT）有关；后者很少被描述。严格的文献综述显示，大约有30例与RT相关的天疱疮，这些患者最初已使用局部和/或全身性类固醇治疗，在某些情况下还给予了氨苯砜或其他几种免疫抑制剂。最常见的潜在癌症类型是乳腺癌。我们在这里介绍了一名63岁的男性患者，该患者在入院前3个月接受了辅助放疗以治疗喉癌。他发生了广泛的皮肤，眼睛和口腔侵蚀。尽管临床表现可与副肿瘤性天疱疮媲美，基于直接免疫荧光建立了皮肤粘膜天疱疮的诊断，显示阳性的细胞表面IgG和离散的C3沉积，在猴食道上具有匹配的细胞表面IgG模式。抗桥粒芯蛋白1和3的血清自身抗体高度阳性。没有发现其他自身抗体，因此排除了副肿瘤性天疱疮。该患者接受了高剂量泼尼松龙的治疗，每天静脉使用剂量高达2 mg / kg的部分泼尼松龙，以及局部消毒剂和IV级类固醇乳膏。为了稳定该病，给予了利妥昔单抗2×1,000 mg，导致临床和血清学缓解长达2年。我们显示，利妥昔单抗代表了难治性RT相关性天疱疮的良好治疗选择，