Wu Xueqiong, Lv Zhenhui, Li Wenjia, Meng Zhaosheng, Wan Shaw P
Zibo Central Hospital.
The People's Hospital of Huantai County, Zibo, Shandong, China.
Medicine (Baltimore). 2020 Dec 4;99(49):e23496. doi: 10.1097/MD.0000000000023496.
Rituximab is a monoclonal antibody directed against B cells and is a first-line agent for the treatment of B cell lymphoma and a second-line agent for the treatment of idiopathic thrombocytopenic purpura (ITP). It has also been used for the treatment of several other autoimmune diseases. Epidermolysis bullosa acquisita (EBA) has never been reported as an adverse effect resulted from rituximab therapy.
A 54-year-old female presented with relapse of the ITP for around eight months. She was treated with rituximab. Intramuscular chlorpheniramine and intravenous methylprednisolone and cimetidine were used as premedication before rituximab infusion. The infusion was initially started at 50 mg/h for 1 h followed by 100 mg/h till the end of infusion. The day after rituximab infusion, the patient noticed pruritic blisters on both arms and chest skin. The next day, the lesions increased in severity and extent.
The skin biopsy established the diagnosis of EBA. H&E staining revealed subepidermal blisters infiltrated by inflammatory cells, including eosinophils and lymphocytes. Direct immunofluorescence (DIF) showed linear deposition of IgG and C3 at the dermoepidermal junction. Indirect immunofluorescence with the patient's serum on salt-split skin revealed exclusive dermal binding of circulating IgG antibasement membrane antibodies at a titer of 1:160.
She was treated with intravenous methylprednisolone and was continued on oral prednisolone.
The lesions regressed. Six weeks later, she had a recurrence of similar lesions but in milder form. This episode subsided in 4 to 5 days with topical steroid application.
Physicians should consider this diagnosis when a patient develops bullous skin eruptions while undergoing Rituximab therapy.
利妥昔单抗是一种针对B细胞的单克隆抗体，是治疗B细胞淋巴瘤的一线药物，也是治疗特发性血小板减少性紫癜（ITP）的二线药物。它也被用于治疗其他几种自身免疫性疾病。获得性大疱性表皮松解症（EBA）从未被报道为利妥昔单抗治疗导致的不良反应。
一名54岁女性，ITP复发约8个月。她接受了利妥昔单抗治疗。在输注利妥昔单抗前，使用肌内注射氯苯那敏、静脉注射甲泼尼龙和西咪替丁作为预处理。输注最初以50mg/h的速度开始1小时，然后以100mg/h的速度持续至输注结束。利妥昔单抗输注后的第二天，患者注意到双臂和胸部皮肤出现瘙痒性水疱。第二天，病变的严重程度和范围增加。
皮肤活检确诊为EBA。苏木精-伊红染色显示表皮下水疱有炎症细胞浸润,包括嗜酸性粒细胞和淋巴细胞。直接免疫荧光（DIF）显示真皮表皮交界处有IgG和C3的线性沉积。用患者血清对盐裂皮肤进行间接免疫荧光检查，显示循环IgG抗基底膜抗体在真皮的特异性结合，滴度为1:160。
她接受了静脉注射甲泼尼龙治疗，并继续口服泼尼松龙。
病变消退。六周后，她再次出现类似病变，但症状较轻。局部应用类固醇后，这一症状在4至5天内消退。
当患者在接受利妥昔单抗治疗时出现大疱性皮肤疹时，医生应考虑这一诊断。
