Selective IgM Deficiency Accompanied with IgG4 Deficiency, Dermal Complications and a Bronchial Polyp

• Ideura Gen

  First Department of Medicine, Shinshu University School of Medicine

• Agematsu Kazunaga

  Department of Infection and Host Defense, Shinshu University School of Medicine

• Komatsu Yoshimichi

  First Department of Medicine, Shinshu University School of Medicine

• Hatayama Orie

  First Department of Medicine, Shinshu University School of Medicine

• Yasuo Masanori

  First Department of Medicine, Shinshu University School of Medicine

• Tsushima Kenji

  First Department of Medicine, Shinshu University School of Medicine

• Hanaoka Masayuki

  First Department of Medicine, Shinshu University School of Medicine

• Koizumi Tomonobu

  First Department of Medicine, Shinshu University School of Medicine

• Fujimoto Keisaku

  First Department of Medicine, Shinshu University School of Medicine

• Kubo Keishi

  First Department of Medicine, Shinshu University School of Medicine

抄録

Background: IgM deficiency is a rare primary immunodeficiency. As few studies of selective IgM deficiency have been reported among the various other types of primary immunodeficiencies, the detailed pathogenesis of this disorder remains to be elucidated.<br> Case Summary: We clinically analyzed a 37-year-old woman who presented with IgM and IgG4 deficiency and ectopic bronchial pneumonia, and investigated immunological functions. Occlusive pneumonia was repeatedly observed in the right S6 area, and bronchoscopy revealed a polyp in the right B6 orifice, which was later identified as a fibroepithelial polyp after transbronchial endoscopic polypectomy. Two months later, pneumonia involving the right inferior lobe developed. Systemic erythema and pigmentation with bleb formation were also observed on the skin, and were thought to be drug-induced exanthema following a biopsy. Serum levels of IgM and IgG4 were extremely low at 3.0mg/dl and less than 2.0mg/dl, respectively. Circulating CD20 positive B cells were mildly reduced and memory B cells were markedly decreased. The majority of B cells expressed IgM on their surface. There were no abnormalities in cell counts of neutrophils, T cells, NK cells and monocytes. Chemotaxins, bactericidal activity and phagocytosis of neutrophils were normal.<br> Discussion: There have been no case reports of selective IgM deficiency with concurrent IgG4 deficiency, various dermal symptoms and a bronchial polyp, as demonstrated in our patient.<br>

収録刊行物

• Allergology International

  Allergology International 57 (1), 99-105, 2008

  一般社団法人日本アレルギー学会

キーワード

• bronchial polyp
• drug-induced exanthema
• immunoglobulin G4 deficiency
• selective immunoglobulin M deficiency

詳細情報

• CRID: 1390282679610518656
• NII論文ID: 10021246249
• NII論文ID: 130004476962
• NII書誌ID: AA11091750
• DOI: 10.2332/allergolint.c-06-52
• ISSN: 14401592
• ISSN: 13238930
• PubMed: 18089938
• Web Site: http://api.elsevier.com/content/article/PII:S1323893015307607?httpAccept=text/xml
• Web Site: http://api.elsevier.com/content/article/PII:S1323893015307607?httpAccept=text/plain
• Web Site: https://search.jamas.or.jp/link/ui/2008307067
• 本文言語コード: en
• データソース種別:
  • JaLC
  • Crossref
  • PubMed
  • CiNii Articles
• 抄録ライセンスフラグ: 使用不可