[Histiocytosis X with partial antehypophyseal deficiencies. 2 cases]

Diabetes insipidus is the most common endocrine disturbance associated with chronic multifocal form of histiocytosis X. Involvement of the hypothalamus can lead to growth hormone deficiency and short stature in children. Two cases with hypogonadism, growth hormone deficiency and diabetes insipidus are reported. In a 22-year-old man plasma testosterone was low and increased after administration of human chorionic gonadotrophin. Cutaneous and lytic bone lesions disappeared after treatment with Vinblastin. Partial diabetes insipidus was treated with clofibrate. A 59-year-old woman was found to have low pituitary gonadotrophins that failed to rise after administration of gonadotropin-releasing hormone and hyperprolactinemia. Fatal outcome was associated with polyuria, bone, cutaneous, gut and hypothalamic infiltration by histiocytes. There was no direct involvement of the anterior pituitary gland.