蛋白A免疫吸附疗法治疗寻常型天疱疮OA
Treatment of pemphigus vulgaris with protein A immunoadsorption
患者女,25岁,全身红斑、水疱、糜烂伴疼痛 4 年余,加重 1 周.4 年前患者因口腔多发溃疡、全身多发红斑和薄壁水疱,确诊为"寻常型天疱疮",随后使用激素、免疫抑制剂及利妥昔单抗等生物制剂等治疗,病情控制不佳,仍反复发作.入院查体:满月面容,水牛背,体质量指数(BMI)28.08.专科查体:全身多发大片红斑、松弛性水疱、糜烂、结痂,腋下、外阴可见大面积红斑、水疱、疣状斑块.天疱疮皮损面积指数(PDAI)评分为49 分.实验室检查:天疱疮抗体:抗-Dsg1 抗体237.8 U/mL,抗-Dsg3 抗体 174.5 U/mL;皮肤分泌物培养多种病原体阳性.诊断:①寻常型天疱疮;②皮肤感染;③药物性库欣综合征等.经多学科会诊以及患者知情同意后,予糖皮质激素、甲氨蝶呤联合蛋白A免疫吸附疗法(PA-IA)治疗(10 次).出院后,患者天疱疮抗体滴度降低,全身皮损好转.后续随访 6 个月,患者无新发皮损,天疱疮抗体滴度持续降低.
A 25-year-old woman presented with a four-year history of widespread erythema,blisters,and erosions,accompanied by pain that had worsened over the past week.Four years ago,she was diagnosed with pemphigus vulgaris(PV)based on multiple oral ulcers,widespread erythema,and thin-walled blisters.Despite treatment with corticosteroids,immunosuppressants,and biologics such as rituximab,her condition remained poorly controlled with recurrent flares.A general examination revealed cushingoid features,a buffalo hump,and a Body Mass Index of 28.08.The dermatological examination showed extensive erythema,flaccid bullae,erosions,and crusting on the scalp,trunk,and extremities,with profuse serous exudate.Erythematous bullous,verrucous plaques were also noted in the axillary and genital areas.The Pemphigus Disease Area Index score was 49.Laboratory tests revealed anti-Dsg1 antibodies at 237.8 U/mL and anti-Dsg3 antibodies at 174.5 U/mL.Skin secretion cultures grew multiple pathogenic organisms.The final diagnoses were pemphigus vulgaris,cutaneous infection,and drug-induced Cushing's syndrome,and others.Following multidisciplinary consultation and informed consent,the patient received corticosteroids,methotrexate,and protein A immunoadsorption(PA-IA)therapy for a total of 10 sessions.After discharge,her autoantibody titers decreased,and skin lesions improved significant-ly.During a 6-month follow-up period,the patient remained lesion-free,with a sustained decline in pemphigus-related autoantibody titers.
张辰希;沈静;张晓颖;郭丽恒;陈佳龙;王琦;曾抗;李常兴
南方医科大学南方医院,广东 广州 510515||东莞市人民医院,广东 东莞 523000南方医科大学南方医院,广东 广州 510515南方医科大学南方医院,广东 广州 510515||东莞市人民医院,广东 东莞 523000南方医科大学南方医院,广东 广州 510515||东莞市人民医院,广东 东莞 523000南方医科大学南方医院,广东 广州 510515南方医科大学南方医院,广东 广州 510515南方医科大学南方医院,广东 广州 510515南方医科大学南方医院,广东 广州 510515||东莞市人民医院,广东 东莞 523000
寻常型天疱疮重症天疱疮免疫吸附治疗皮肤感染
pemphigus vulgarissevere pemphigusimmunoadsorption therapycutane-ous infection
《皮肤性病诊疗学杂志》 2026 (1)
35-41,7
国家自然科学基金(82173437)
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