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Hand-Schüller-Christian disease with occult diabetes insipidus, cardiac failure and renal dysfunction
1Second Department of Internal Medicine, Tohoku University School of Medicine, Sendai, Japan.
Insights
A 60-year-old man with Hand-Schüller-Christian disease presented with xanthogranuloma and unusual diabetes insipidus. Vincristine effectively treated the xanthogranuloma, while diabetes insipidus management was guided by patient symptoms.
Area of Science:
- Endocrinology
- Dermatology
- Oncology
Background:
- Hand-Schüller-Christian disease is a rare Langerhans cell histiocytosis variant.
- Classic triad includes bone defects, diabetes insipidus, and exophthalmos.
- This case highlights unusual complications and treatment responses.
Observation:
- A 60-year-old male diagnosed with Hand-Schüller-Christian disease.
- Presented with facial and nuchal xanthogranuloma, exophthalmos, bone decalcification, and ADH-resistant diabetes insipidus.
- Skin biopsy confirmed eosinophilic granuloma cell infiltration.
Findings:
- The patient exhibited ADH-resistant diabetes insipidus with normal urine volume, suggesting renal dysfunction.
- Hypophyseal, adrenal, and thyroid functions were normal.
- Vincristine treatment led to xanthogranuloma regression.
Implications:
- Vincristine shows efficacy in managing xanthogranuloma in Hand-Schüller-Christian disease.
- The case underscores the importance of individualized management for diabetes insipidus based on symptoms.
- Highlights the complex interplay of endocrine, renal, and cardiac complications in this rare disease.
Abstract:
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.