Case 1: A 35-year-old woman presented with diabetes insipidus diagnosed at the age of six years. Ten years after diagnosis, she presented with facial sinus infiltration, pleural thickening, long bone lesions and retroperitoneal infiltration. An unusual well delimited tissular perisplenic infiltration with splenic deformation suggesting fibrosis on imaging (Fig. 1A–D) was also visible. The sinus biopsy led to a diagnosis. The patient received several successive treatments, including vinblastine and corticosteroids, interferon-α, anakinra, infliximab, and methotrexate.
