Bullous Pemphigoidに合併した膜性じん症の一例

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タイトル別名
  • Membranous Glomerulonephritis associated with Bullous Pemphigoid
  • Bullous Pemphigoid ニ ガッペイシタ マク セイ ジンショウ

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We describe a 39-year-old woman who was admitted to our hospital because of bullous pemphigoidd associated with membranous glomerulonephritis. An immunofluorescent staining of the skin biopsy specimen showed linear deposition of IgG, C3 and fibrinogen along the basement membrane zone. Patient's serum contained anti-basement membrane zone antibody and showed positive staining up to 1:1024 inn dilution as revealed by indirect immunofluorescent technique. Six months later, she manifested nephrotic syndrome. Renal biopsy specimen showed membranous glomerulonephritis (Stage I, Churg J.) with fine granular deposition of IgG and C3 along glomerular capillary loops. Anti-complementary activity of the serum was negative and Clq binding activity was within normal limits. FITC-labeled patient's 1-globulin failed to stain normal and patient's renal spe cimens and acid-eluted patient's renal specimens, demonstrating that patient's serum did not containn anti-glomerular basement membrane antibody and skin basement membrane zone antigens did not deposit in the glomeruli. Membranous glomerulonephritis is considered as immune complex disease andd bullous pemphigoid also as autoimmune disease. Both diseases may be mediated by the same fundamental immunological mechanism. However, immunological relationship of both diseases could not be proved in our patient, although this association is very interesting to speculate the pathogenesis of the immunological disorders.

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