Central Diabetes Insipidus as the Inaugural Manifestation of Langerhans Cell Histiocytosis in Adult

Abstract

Langerhans histiocytosis (LH) is a rare disease that is more prevalent in children. Adult onset LH is less observed. Its etiology still remains unclear. Bone involvement is the most frequent. Other organs involvement such as pulmonary, pituitary and cutaneous is rarer. Diagnosis confirmation relies on histological examination. It highlights the accumulation of Langerhans cells organized as granulomas with positive immunohistochimical staining for CD1a. Optimal treatment choices are still undefined. It depends whether it involve a single system or is a multisystem form with life-threatening organ involvement. Treatment is based on corticosteroids and Vinblastine associated with possibly resorting to surgery in some cases. In this report, we present the case of a male patient who developed multisystem LH (MS-LH) with multiple bone lesions, pulmonary involvement and pituitary infiltration. This patient was successfully treated by local surgical curettage and adjuvant corticoids therapy.