[A female patient with earache, polyuria and polydipsia]

Langerhans cell histiocytosis is a rare inflammatory bone marrow neoplasia that frequently affects bone, lung, skin and pituitary gland. Due to its broad spectrum of clinical presentation, an appropriate diagnosis might be difficult.

A 54-year-old female patient complained of pain in her right ear for 5 months. On account of similar complaints, a mastoidectomy had already been performed 3 years ago. Histology at that time revealed nonspecific inflammation. Furthermore, she reported excessive thirst.

Computed tomography of the temporal bones showed osteolysis in the mastoid. Magnetic resonance imaging and bone scintigraphy assessed these changes as uncharacteristically inflammatory. Polydipsia proved to be a symptom of central diabetes insipidus in the water deprivation test. Finally, remastoidectomy provided histologic evidence of Langerhans cell histiocytosis.

Besides systemic chemotherapy with cytarabine, the patient also received denosumab and desmopressin.

Langerhans cell histiocytosis involving cranial bones is often associated with diabetes Insipidus. Knowledge about the distinctive constellation may lead to a more rapid diagnosis and improved prognosis.