Background: In pemphigus, loss of epidermal adhesion is induced by binding of circulating autoantibodies to the desmosomal cadherins desmoglein 3 (Dsg3) in pemphigus vulgaris (PV) and desmoglein 1 (Dsg1) in pemphigus ...Background: In pemphigus, loss of epidermal adhesion is induced by binding of circulating autoantibodies to the desmosomal cadherins desmoglein 3 (Dsg3) in pemphigus vulgaris (PV) and desmoglein 1 (Dsg1) in pemphigus foliaceus (PF), respectively. Therapeutic removal of Dsg- reactive autoantibodies by immunoadsorption (IA) has been demonstrated to exert clinical remission of the disease. Objectives: The aim of this intervention study was to evaluate the efficacy and safety of the peptide- based Globaffin adsorber system in the treatment of severe pemphigus cases. Patients and Methods: We applied IA in 4 PV and 2 PF patients with severe chronic disease resistant to conventional immunosuppressive therapy. IA was performed on 4 consecutive days, representing 1 treatment cycle, followed by a 4- week treatment- free interval. Serum samples for determining serum IgG and anti- Dsg1Dsg3 IgG autoantibodies were drawn daily before and after IA, respectively. During follow- up, patients were examined carefully, and laboratory parameters were controlled monthly for up to 1 year. Results: IA led to excellent clinical responses. Skin and mucosal lesions cleared almost completely within weeks. One IA cycle reduced anti- Dsg1 and anti- Dsg3 autoantibodies by an average of 50- 70% as determined by ELISA. Conclusions: Using the Globaffin adsorber system, IA represents an effective and safe treatment opportunity in severe and therapy- resistant pemphigus.展开更多
患儿 男,3日龄,因＂躯干、四肢部分皮肤破溃、糜烂3d＂于2016年7月就诊于四川大学华西医院.患儿3d前即出生时被发现头面部、四肢及腹股沟处皮肤部分破溃、糜烂,系第1胎第1产,自然出生,出生胎龄36周,生后l、5 min Apgar评分均为10分,产...患儿 男,3日龄,因＂躯干、四肢部分皮肤破溃、糜烂3d＂于2016年7月就诊于四川大学华西医院.患儿3d前即出生时被发现头面部、四肢及腹股沟处皮肤部分破溃、糜烂,系第1胎第1产,自然出生,出生胎龄36周,生后l、5 min Apgar评分均为10分,产程中无外伤.其母36岁,2016年4月因＂口腔糜烂1个月＂于华西口腔医院就诊,其时孕24周,未行病理活检,于我院完善外周血抗桥粒芯糖蛋白（Dsg）l抗体和3抗体检测,分别为1.69 x 10^5 U/L（＞0.15 x 10^5U/L为阳性）、1.97x 10^5 U/L（＞0.1x10^5 U/L为阳性）,诊断为＂寻常型天疱疮＂,并接受口服泼尼松（30 rr/d）治疗,口腔病损有所控制,其后不规则服药,其母亲孕期产前检查包括梅毒等性病检查无异常.展开更多
文摘Background: In pemphigus, loss of epidermal adhesion is induced by binding of circulating autoantibodies to the desmosomal cadherins desmoglein 3 (Dsg3) in pemphigus vulgaris (PV) and desmoglein 1 (Dsg1) in pemphigus foliaceus (PF), respectively. Therapeutic removal of Dsg- reactive autoantibodies by immunoadsorption (IA) has been demonstrated to exert clinical remission of the disease. Objectives: The aim of this intervention study was to evaluate the efficacy and safety of the peptide- based Globaffin adsorber system in the treatment of severe pemphigus cases. Patients and Methods: We applied IA in 4 PV and 2 PF patients with severe chronic disease resistant to conventional immunosuppressive therapy. IA was performed on 4 consecutive days, representing 1 treatment cycle, followed by a 4- week treatment- free interval. Serum samples for determining serum IgG and anti- Dsg1Dsg3 IgG autoantibodies were drawn daily before and after IA, respectively. During follow- up, patients were examined carefully, and laboratory parameters were controlled monthly for up to 1 year. Results: IA led to excellent clinical responses. Skin and mucosal lesions cleared almost completely within weeks. One IA cycle reduced anti- Dsg1 and anti- Dsg3 autoantibodies by an average of 50- 70% as determined by ELISA. Conclusions: Using the Globaffin adsorber system, IA represents an effective and safe treatment opportunity in severe and therapy- resistant pemphigus.
文摘患儿 男,3日龄,因＂躯干、四肢部分皮肤破溃、糜烂3d＂于2016年7月就诊于四川大学华西医院.患儿3d前即出生时被发现头面部、四肢及腹股沟处皮肤部分破溃、糜烂,系第1胎第1产,自然出生,出生胎龄36周,生后l、5 min Apgar评分均为10分,产程中无外伤.其母36岁,2016年4月因＂口腔糜烂1个月＂于华西口腔医院就诊,其时孕24周,未行病理活检,于我院完善外周血抗桥粒芯糖蛋白（Dsg）l抗体和3抗体检测,分别为1.69 x 10^5 U/L（＞0.15 x 10^5U/L为阳性）、1.97x 10^5 U/L（＞0.1x10^5 U/L为阳性）,诊断为＂寻常型天疱疮＂,并接受口服泼尼松（30 rr/d）治疗,口腔病损有所控制,其后不规则服药,其母亲孕期产前检查包括梅毒等性病检查无异常.
