Severe pemphigus vulgaris: successful combination therapy of plasmapheresis followed by intravenous high-dose immunoglobulin to prevent rebound increase in pathogenic IgG.
A case of severe pemphigus vulgaris (PV), which did not respond to pulse therapy with intravenous (IV) methylprednisolone (1,000 mg/day for 3 days), but was successfully treated with a combination of double-filtration plasmapheresis (DFPP), immediately followed by high-dose IVIg (20 g/day for 5 days), eventually leading to suppression of the rebound increase in pathogenic PV-IgG for 12 months, is reported. Weekly enzyme-linked immunosorbent assay for desmoglein (Dsg) 1 and Dsg3 demonstrates a distinct difference in the alteration curves of serum levels of pathogenic IgG (anti-Dsg1 and Dsg3 antibodies) after DFPP with and without high-dose IVIg. Our experience suggests that combination therapy of DFPP with high-dose IVIg is effective for pathogenic PV-IgG removal and prevention of feedback rebound increases in pathogenic PV-IgG, leading to a long-term amelioration of clinical blistering in the present case.
Aoyama
Yumi
Y
Department of Dermatology, Gifu University School of Medicine, Yanagido 1-1, Gifu City, Japan. ymaoyama@gifu-u.ac.jp
Nagasawa
Chikako
C
Nagai
Miki
M
Kitajima
Yasuo
Y
D003131:Combined Modality Therapy; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood* / Q000276:immunology; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage*; D007155:Immunologic Factors / Q000008:administration & dosage*; D008297:Male; D008875:Middle Aged; D010392:Pemphigus / Q000097:blood* / Q000276:immunology / Q000628:therapy*; D010956:Plasmapheresis; D012074:Remission Induction; D012720:Severity of Illness Index
D007074:Immunoglobulin G; D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors