Rowell综合征2例临床分析

Clinical analysis of Rowell's syndrome in 2 cases

目的分析2例Rowell综合征患者临床资料,探讨其临床病理特点及治疗转归。方法回顾性分析2022年8月广东医科大学附属医院2例Rowell综合征患者的临床资料,包括临床表现、实验室检查、皮损组织病理检查、诊断及治疗转归情况。结果2例皮损均表现为泛发性靶形皮损,1例双耳冻疮样红斑,1例盘状红斑;皮损组织直接免疫荧光检查均为阴性。2例患者抗核抗体、抗SSA/Ro抗体均为阳性。2例皮损组织病理表现均有角化过度,表皮轻度增生,可见表皮部分坏死,较多角化不良细胞,基底细胞液化变性;真皮浅层血管周围可见淋巴细胞浸润。1例因处于妊娠期住院期间给予泼尼松(25 mg/次,1次/d)、羟氯喹(0.2 g/次,2次/d)口服,5 d后终止妊娠,1个月后给予泼尼松(50 mg/次,1次/d)、羟氯喹(0.2 g/次,2次/d)口服,病情稳定后常规减少糖皮质激素用量;1例患者住院期间给予泼尼松(60 mg/次,1次/d)、羟氯喹(0.2 g/次,2次/d)口服,治疗好转后出院;随访结束时2例患者均病情稳定。结论Rowell综合征皮损特征为红斑狼疮伴多形性红斑样病变,免疫学指标缺乏特异性,组织病理表现与多形性红斑、红斑狼疮相似,足量糖皮质激素联合羟氯喹治疗效果良好。

Objective To analyze the clinical data of 2 patients with Rowell's syndrome,and to investigate the clinicopathological features,treatment and prognosis.Methods The clinical data of 2 patients with Rowell's syndrome who were admitted to the Affiliated Hospital of Guangdong Medical University in August 2022 were retrospectively analyzed,including clinical manifestations,laboratory examinations,pathological examinations,diagnosis,treatment and prognosis.Results Two patients were clinically manifested with generalized targeted lesions,including chilblain lesions on two ears of one patient,and discoid lesion in the other patient,with negative direct immunofluorescence results in lesions.ANA and anti-SSA/Ro antibodies were positive in 2 patients.The pathological examinations of lesions in 2 patients showed hyperkeratiniztion,mild hyperplasia of epidermis,partial necrosis of keratinocyte,large amount of dyskeratocytes,basal cell liquefaction,and perivascular infiltration of lymphocytes in the upper dermis.One patient was given oral administration of prednisone(25 mg/time,once a day)and hydroxychloroquine(0.2 g/time,twice a day)during hospitalization because of pregnancy;she was terminated the pregnancy 5 days later,and was given prednisone(50 mg/time,once a day)and hydroxychloroquine(0.2 g/time,twice a day)orally;and the glucocorticoid dosage was reduced routinely when the disease was stable.The other patient was given oral administration of prednisone(60 mg/d,once a day)and hydroxychloroquine(0.2 g/time,twice a day),and he was discharged after improvement.The disease was stable at the end of follow-up in both patients.Conclusions Rowell's syndrome is characterized by the combination of lupus erythematosus and erythema multiforme-like lesions,and the immune indexes lack specificity.The histopathological manifestations are similar to those of erythema multiforme and lupus erythematosus,and the combination of sufficient glucocorticoids and hydroxychloroquine is effective.