ECE2013 Poster Presentations Neuroendocrinology (42 abstracts)
Central diabetes insipidus, as the first sign of Langerhans cell histiocytosis in an adult
Nerea Egaña 1 , Irune Ruiz 2 , Ismene Bilbao 1 , Maite Aramburu 1 , Alfredo Yoldi 1 , Cristina Garcia 1 , Mariano Alvarez Coca 1 , Maria Luisa Antuñano 1 & Miguel Goena 1
1Endocrinology. Hospital Donostia, San Sebastian, Spain, 2Pathology. Hospital Donostia, San Sebastian, Spain.
Introduction: Langerhans cell histiocytosis (LCH) is a rare granulomatous disease of unknown etiology. LCH may be localized or be a systemic disease. The diagnosis is frecuent in pediatric age. In adults, infiltration is most frecuently in bones, lungs and skin, and shows particular predilection for hypothalamo-pituitary axis.
Case: A 51 years old man was referred due to polyuria of ~15 l of daily urine output. His past medical history revealed bronchiectasis and skin problems. Laboratory test demonstrated hypernatremia, decreased urinary and increased serum osmolarities. Water deprivation test was not held due to severe signs of dehydration. Pituitary MRI showed enhancing pituitary signal with thickened stalk and loss of the bright spot of the neurohypophysis. Desmopressin was started with a presumptive diagnosis of insipidus diabetes and urine output and biochemical tests returned to normal. Anterior pituitary hormones were normal. Months later, he presents temporal cephalea and MRI revealed two subcortical lesions. Neurosurgery was practiced and histology confirmed Langerhans cell proliferation. His skin and lungs were also affected. Levothyroxine and testosterone therapy were commenced due to progressive failure of the gonadal and the thyroid axis. Nowadays, he has begun chemotherapy and corticotherapy.
Conclusions: i) Although LCH is mainly considered a pediatric disease, it may be diagnosed in adults, ii) the difficulty in making an accurate diagnosis is reflected in the long time that takes from the onset of the symtoms to the diagnosis, due to the fact that patients visit many different specialistes, the lack of clinical suspicion, and the variable characteristics of the disease, and iii) LCH is a multisystemic disease and multidisciplinary management is important.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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