title: Hypocomplementemic urticarial vasculitis syndrome is associated with high levels of serum IgG4: a clinical manifestation that mimics IgG4-related disease.
abstract: A 58-year-old Japanese woman presented with recurrent abdominal pain, chronic urticaria, and petechiae on her extremities, and hypocomplementemia, findings that were consistent with hypocomplementemic urticarial vasculitis syndrome (HUVS). A laboratory examination revealed that she had markedly elevated IgG levels (4,448 mg/dL; normal range, 870-1,700 mg/dL) with particularly high IgG4 levels (1,050 mg/dL; normal range, 48-105 mg/dL) and a high IgG4/total IgG ratio (0.22; normal range, 0.02-0.05). A skin biopsy demonstrated leukocytoclastic vasculitis with IgG4 deposition in the vascular lumen and vascular walls. A lymph node biopsy revealed reactive lymphoid hyperplasia with numerous IgG4-positive cells in the perifollicular area, but no sclerotic findings. A chromosomal analysis of an enlarged lymph node, without phytohemagglutinin (PHA) stimulation, demonstrated that one in every three analyzed cells had abnormalities, such as 44, XX, -13, add(15)(p11), -17, -17, and mar.
authors.affiliation: Department of Internal Medicine, Department of Clinical Pathology, Aichi Medical University School of Medicine, Japan.
authors.forename: Ryo
authors.initials: R
authors.lastname: Wakamatsu
authors.forename: Hitoshi
authors.initials: H
authors.lastname: Watanabe
authors.forename: Keisuke
authors.initials: K
authors.lastname: Suzuki
authors.forename: Norihiro
authors.initials: N
authors.lastname: Suga
authors.forename: Wataru
authors.initials: W
authors.lastname: Kitagawa
authors.forename: Naoto
authors.initials: N
authors.lastname: Miura
authors.forename: Kazuhiro
authors.initials: K
authors.lastname: Nishikawa
authors.forename: Toyoharu
authors.initials: T
authors.lastname: Yokoi
authors.forename: Syogo
authors.initials: S
authors.lastname: Banno
authors.forename: Hirokazu
authors.initials: H
authors.lastname: Imai
mesh_terms: D002869:Chromosome Aberrations; D003165:Complement System Proteins / Q000172:deficiency*; D005260:Female; D006801:Humans; D006942:Hypergammaglobulinemia / Q000097:blood / Q000150:complications* / Q000276:immunology*; D007074:Immunoglobulin G / Q000097:blood*; D008875:Middle Aged; D013577:Syndrome; D014581:Urticaria / Q000097:blood / Q000150:complications* / Q000235:genetics / Q000276:immunology*; D014657:Vasculitis / Q000097:blood / Q000150:complications* / Q000235:genetics / Q000276:immunology*; D018366:Vasculitis, Leukocytoclastic, Cutaneous / Q000097:blood / Q000150:complications / Q000235:genetics / Q000276:immunology
chemical_list: D007074:Immunoglobulin G; D003165:Complement System Proteins