Endocrine manifestations of Erdheim-Chester disease (a distinct form of histiocytosis).
Erdheim-Chester disease (ECD) is a disorder of unclear aetiology, characterized by exuberant histiocyte proliferation and a variable clinical course. We report the case of a woman with multi-organ involvement secondary to ECD. Central diabetes insipidus (CDI), hyperprolactinaemia, gonadotropin insufficiency and decreased insulin-like growth factor I levels were present, suggesting hypothalamic-pituitary dysfunction. The high-intensity signal of the posterior pituitary on T1-weighted images was absent on magnetic resonance imaging, but no sellar mass lesions or stalk thickening were apparent. Additionally, our patient had bilateral adrenal enlargement. Even though ECD is a rare cause of neuroendocrine dysfunction or adrenal enlargement, it should be considered in patients with these disorders in the setting of multiorgan disease.
Tritos
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Division of Endocrinology, Beth Israel Deaconess Medical Center, Boston, Massachusetts, USA.
Weinrib
S
S
Kaye
T B
TB
D003919:Diabetes Insipidus / Q000209:etiology; D004700:Endocrine System Diseases / Q000209:etiology*; D005260:Female; D006062:Gonadotropins / Q000172:deficiency; D015614:Histiocytosis / Q000150:complications* / Q000503:physiopathology; D006801:Humans; D006966:Hyperprolactinemia / Q000209:etiology; D007030:Hypothalamo-Hypophyseal System / Q000503:physiopathology; D007334:Insulin-Like Growth Factor I / Q000032:analysis; D008875:Middle Aged
D006062:Gonadotropins; D007334:Insulin-Like Growth Factor I