Pfütze Martin, Eming Rüdiger, Kneisel Andrea, Kuhlmann Uwe, Hoyer Joachim, Hertl Michael
Department of Dermatology and Allergology, Philipps University, Marburg, Germany.
Dermatology. 2009;218(3):237-45. doi: 10.1159/000187431. Epub 2008 Dec 16.
Pemphigus vulgaris (PV) is a life-threatening autoimmune blistering skin disease which is associated with pathogenic IgG autoantibodies against desmogleins (Dsg) 1 and 3. Novel therapeutic strategies such as immunoadsorption (IA) or the anti-CD20 antibody rituximab (Rtx) hold promise to be effective in severe or recalcitrant PV.
In the present retrospective study, 6 patients with extensive cutaneous PV were subjected to adjuvant IA treatment while 5 patients with severe mucosal PV received adjuvant Rtx treatment.
Within 6 months, IA and Rtx induced excellent clinical responses which were associated with a significant reduction of prednisolone doses and a decrease in anti-Dsg-specific IgG. Over a 12-month period, 3 IA-treated patients required additional adjuvant drugs while all of the PV patients on Rtx had no or only minimal residual symptoms.
The relative therapeutic (long-term) efficacy of IA and Rtx in cutaneous versus mucosal PV needs to be evaluated in a prospective study.
寻常型天疱疮（PV）是一种危及生命的自身免疫性水疱性皮肤病，与抗桥粒芯糖蛋白（Dsg）1和3的致病性IgG自身抗体有关。免疫吸附（IA）或抗CD20抗体利妥昔单抗（Rtx）等新型治疗策略有望有效治疗重度或难治性PV。
在本回顾性研究中，6例广泛皮肤型PV患者接受辅助IA治疗，5例重度黏膜型PV患者接受辅助Rtx治疗。
6个月内，IA和Rtx均诱导出良好的临床反应，这与泼尼松龙剂量显著减少及抗Dsg特异性IgG降低有关。在12个月期间，3例接受IA治疗的患者需要额外的辅助药物，而所有接受Rtx治疗的PV患者均无或仅有轻微残留症状。
IA和Rtx在皮肤型与黏膜型PV中的相对治疗（长期）疗效需要在前瞻性研究中进行评估。
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