Langerhans cells histiocytosis (LCH) is a disease caused by the proliferation and abnormal accumulation of Langerhans cells in different tissues and organs. It is a rare disease that still hides many of its mysteries as to its etiology and pathophysiology. The cerebral localization remains exceptional and dominated by hypothalamic-pituitary axis involvement. We report a case of a 20-year-old patient followed for acquired central diabetes insipidus with Panhypopituitarism and multiple dermatological lesions, who recently had visual acuity decline with progressive cerebellar syndrome in a state of apyrexia. Brain MRI showed multiples scattered plaques and nodular lesions involving the periventricular white matter, the basal ganglia, cerebellum, brainstem and cervical medulla. The two diagnoses suggested in this clinical presentation sarcoidosis with pituitary localization and cerebral Langerhans cells histiocytosis. A skin biopsy confirmed the diagnosis of langherans cell histiocytosis.
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