小剂量利妥昔单抗联合糖皮质激素治疗儿童落叶型天疱疮一例疗效观察

Efficacy of low-dose rituximab combined with glucocorticoids in a case of childhood pemphigus foliaceus:a clinical observation

患儿男,9岁,头皮红斑、糜烂伴瘙痒1个月,泛发全身1周。皮损组织病理:表皮内颗粒层下方水疱形成,疱内见大量棘层松解细胞,真皮浅层小血管周围淋巴细胞及嗜酸性粒细胞浸润。直接免疫荧光检查示表皮细胞间IgG、补体C3呈网状沉积,IgM、IgA阴性。酶联免疫吸附实验检测血清抗Dsg1抗体阳性(157.00 U/ml)。诊断:落叶型天疱疮。入院后应用泼尼松40 mg/d疗效欠佳,改用小剂量利妥昔单抗(每周100 mg,共4周)联合泼尼松(20 mg/d)治疗,病情控制良好。泼尼松逐渐减量至7.5 mg/d维持治疗,随访24个月病情无复发。

A 9-year-old boy presented with pruritic erythema and erosions of the scalp for 1 month,which spread all over the body for 1 week.Histopathological examination of the skin lesions showed blisters forming under the granular layer of the epidermis,a large number of acantholytic cells in the blisters,and perivascular infiltration of lymphocytes and eosinophils in the superficial dermis.Direct immunofluorescence study showed reticular deposition of IgG and complement C3 between epidermal cells,and negative staining for IgM and IgA.Enzyme-linked immunosorbent assay showed the presence of serum anti-Dsg1 antibody(157.00 U/ml).The patient was diagnosed with pemphigus foliaceus.After admission,the patient showed poor response to the treatment with prednisone at a dose of 40 mg/d.After the treatment with low-dose rituximab(100 mg per week,4 weeks)combined with prednisone(20 mg/d),her condition was well controlled.The dosage of prednisone gradually decreased to 7.5 mg/d for maintenance treatment,and no recurrence was observed during 24-month follow-up.