Hand-Schueller-Christian disease with occult diabetes insipidus, cardiac failure and renal dysfunction.

  • KIMURA Tokihisa
    The Second Department of Internal Medicine, Tohoku University School of Medicine
  • OTA Kozo
    The Second Department of Internal Medicine, Tohoku University School of Medicine
  • SHOJI Masaru
    The Second Department of Internal Medicine, Tohoku University School of Medicine
  • INOUE Minoru
    The Second Department of Internal Medicine, Tohoku University School of Medicine
  • SATO Kazutoshi
    The Second Department of Internal Medicine, Tohoku University School of Medicine
  • OHTA Masahiro
    The Second Department of Internal Medicine, Tohoku University School of Medicine
  • YAMAMOTO Tadasu
    The Second Department of Internal Medicine, Tohoku University School of Medicine
  • ENDO Kazuyasu
    The Second Department of Internal Medicine, Tohoku University School of Medicine
  • OSAKI Hirofumi
    The Second Department of Internal Medicine, Tohoku University School of Medicine
  • AOYAMA Hiroaki
    The Department of Dermatology, Tohoku University School of Medicine
  • ABE Keishi
    The Second Department of Internal Medicine, Tohoku University School of Medicine
  • YOSHINAGA Kaoru
    The Second Department of Internal Medicine, Tohoku University School of Medicine

抄録

A 60-year-old man was diagnosed as Hand-Schüller-Christian disease due to the triad of exophthalmus, decalcification of the bone, and diabetes insipidus. He had xanthogranuloma on the face and a nuchal region, and unusual complications of ADH-resistant diabetes insipidus due to renal dysfunction, and chronic cardiac failure. Urine osmolality was hypotonic, but urine volume was within the normal limit, despite the presence of central diabetes insipidus. Hypophyseal, adrenal and thyroid function were not remarkable. The skin biopsy showed the infiltration of eosinophilic granuloma cells. Treatment with vincristine was effective to regress the xanthogranuloma. Diabetes insipidus was not treated because of the absence of polyuria and polydipsia.

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