Langerhans Cell Histiocytosis of the Temporal Bone in a Child with Central Diabetes Insipidus

Abstract

Langerhans cell histiocytosis is a rare clonal disease defined by an accumulation of dendritic cells with Langerhans cell immunological properties within different organs of the body. We intend to describe a new case of Langerhans cell histiocytosis of the temporal bone in a child with central diabetes insipidus. The child presented the polyuria-polydipsia syndrome for 6 months with Chronic bilateral otorrhea. On physical examination,there were bilateral retroauricular redness and induration. The otoscopy visualized inflammatory polyps filling all of the right and left external ear canal. Biological exploration confirmed the central diabetes insipidus. Magnetic resonance imaging of the brain showed the presence of pituitary infiltration. Computed tomography showed osseous lysis of the temporal bone bilaterally. A retroauricular biopsy was performed under general anesthesia. Histological and immunohistochemical evaluation confirmed the diagnosis of Langerhans cell histiocytosis