Department of Dermatology, Taizhou Central Hospital (Taizhou University Hospital), Taizhou, China.
Department of Dermatology, Zhong Da Hospital, Southeast University, Nanjing, China.
J Clin Apher. 2023 Aug;38(4):491-494. doi: 10.1002/jca.22036. Epub 2022 Dec 29.
Pemphigus vulgaris (PV) is a chronic, mucocutaneous, autoimmune bullous disease. Double filtration plasmapheresis (DFPP) may be effective when PV fails to be controlled by conventional corticosteroid treatment. The patient was a 64-year-old man with erythema, blisters, and erosions on his head, face, mouth, trunk, limbs, and scrotum for over a month. He was diagnosed with severe PV, and the original rash area continued to expand after treatment with systemic corticosteroids, immunosuppressants, and intravenous immunoglobulin, with massive exudate and ≥5 new blisters and macules still occurring daily. Subsequently, the patient completed three sessions of DFPP. After the first DFPP, the original erosion surface exudate was significantly reduced and gradually healed. After the second DFPP, the erosion area and exudate increased compared with the previous one. After the third DFPP, the rash did not improve further and had a tendency to continue to progress. During the entire three sessions of DFPP, the patient had new blisters and bullae on his limbs every day. The Nikolsky's sign of the limbs turned negative at the initial stage, and then the trunk and limbs Nikolsky's sign became positive again. The titer of autoantibodies did not decrease significantly after the plasmapheresis. The patient eventually died of secondary lung infection and septic shock. The efficacy of DFPP in this patient with refractory severe PV was poor.
寻常型天疱疮（PV）是一种慢性黏膜-皮肤自身免疫性大疱病。当 PV 经常规皮质类固醇治疗不能控制时，双重滤过血浆置换（DFPP）可能有效。患者为 64 岁男性，头面部、口腔、胸腹部、四肢及阴囊红斑、水疱、糜烂 1 个多月。诊断为重症 PV，经系统应用皮质类固醇、免疫抑制剂、静脉注射免疫球蛋白治疗后原皮损面积仍继续扩大，大量渗出且每天仍有≥5 个新发水疱及斑疹。后患者行 3 次 DFPP 治疗。第 1 次 DFPP 后，原糜烂面渗出明显减少，逐渐愈合；第 2 次 DFPP 后，与前 1 次比较，糜烂面积及渗出增加；第 3 次 DFPP 后，皮疹无进一步改善，且有继续进展趋势。在整个 3 次 DFPP 治疗过程中，患者每天四肢均有新发水疱及大疱。四肢尼氏征在初始阶段转为阴性，而后躯及四肢尼氏征再次转为阳性。血浆置换后自身抗体滴度无明显下降。患者最终死于继发性肺部感染和感染性休克。该难治性重症 PV 患者 DFPP 疗效不佳。
