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Endocrine manifestations of Erdheim–Chester disease (a distinct form of histiocytosis)
Author(s) -
Tritos N. A.,
Tritos N. A.,
Weinrib S.,
Kaye T. B
Publication year - 1998
Publication title -
journal of internal medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.625
H-Index - 160
eISSN - 1365-2796
pISSN - 0954-6820
DOI - 10.1111/j.1365-2796.1998.00389.x
Subject(s) - medicine , erdheim–chester disease , diabetes insipidus , histiocytosis , posterior pituitary , endocrine system , magnetic resonance imaging , pituitary stalk , pathology , hyperprolactinaemia , pituitary gland , disease , endocrinology , hormone , radiology , prolactin
. Tritos NA, Weinrib S, Kaye TB (Lahey Hitchcock Clinic, Burlington; Beth Israel Deaconess Medical Center, Boston; and Joslin Diabetes Center, Boston, Massachusetts, USA). Endocrine manifestations of Erdheim–Chester disease (a distinct form of histiocytosis) (Case Report). J Intern Med 1998; 244 : 529–35. 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.