HYPOTHALAMIC-PITUITARY HISTIOCYTOSIS SEEMS LIKE A REAL TUMOR REVEALED BY DIABETES INSIPIDUS: A CASE REPORT

Introduction: The etiological diagnosis of central diabetes insipidus (DI) is difficult when initial imaging by (MRI) is inconclusive, the association with multisystem lesions should suggest a granulomatous origin.We report a case of Langerhansian histiocytosis revealed by diabetes insipidus.

Observation:A 52-year-old woman was referred to our department for the management of a polyuria-polydipsia syndrome, presenting with sub mammary intertrigo, papilo-squamous lesions on the scalp, inflammatory and ulcerated perianal and vaginal lesions and oral ulcerations. The biological work-up showed hypernatremia, central hypothyroidism, hypogonadotropic hypogonadism and an inflammatory syndrome.The morphological work-up showed a hypothalamic process, a cystic and micronodular pulmonary lesion, a significant peri-renal infiltration with a hairy appearance suggestive of histiocytosis.The skin biopsy concluded a Langerhans cell histiocytosis CD1a+, PS100+, CD163-

The patient was treated with vinblastine and corticotherapy in association with desmopressin and levothyroxine with a good clinical evolution without recovery of the endocrine involvement.

Discussion/Conclusion:Langerhansian histiocytosis is a rare systemic disease of adults. Characterized by infiltration of various organs by CD1a+ histiocytes. Diabetes insipidus, secondary to an infiltration of the post pituitary gland, is the most frequent endocrine disorder that generally precedes the other manifestations of the disease, its diagnosis requires a very meticulous etiological investigation and a regular re-evaluation in order not to miss this pathology.