title: Cerebral and Cutaneous Involvements of Xanthoma Disseminatum Successfully Treated with an Interleukin-1 Receptor Antagonist: A Case Report and Minireview.
abstract: A young male presented with panhypopituitarism (including diabetes insipidus) and temporal lobe epilepsy. A histology specimen of cutaneous papules was diagnostic of non-Langerhans histiocytosis. The diagnosis of xanthoma granulomata was considered based on the clinical and brain MRI findings. Brain lesions significantly worsened over time despite radiotherapy until anakinra induced a complete clinical and radiological remission of all active lesions. Although a single case, the outcome of this patient with xanthoma disseminatum treated with an interleukin-1 receptor antagonist opens and strengthens new and recent physiopathogenic and treatment perspectives for the otherwise difficult-to-treat non-Langerhans cell histiocytosis. Similar results with anakinra have been observed in patients with Erdheim-Chester disease and in multicentric reticulohistiocytosis.
authors.affiliation: Departamento de Neurologx00ED;a, Clx00ED;nica Las Condes, Santiago, Chile.
authors.forename: Mario
authors.initials: M
authors.lastname: Campero
authors.forename: Sebastián
authors.initials: S
authors.lastname: Campero
authors.forename: Julia
authors.initials: J
authors.lastname: Guerrero
authors.forename: Achille
authors.initials: A
authors.lastname: Aouba
authors.forename: Alex
authors.initials: A
authors.lastname: Castro
mesh_terms: D000328:Adult; D018501:Antirheumatic Agents / Q000627:therapeutic use*; D004833:Epilepsy, Temporal Lobe / Q000000981:diagnostic imaging / Q000209:etiology; D015616:Histiocytosis, Non-Langerhans-Cell / Q000150:complications / Q000188:drug therapy*; D006801:Humans; D007018:Hypopituitarism / Q000000981:diagnostic imaging / Q000209:etiology; D053590:Interleukin 1 Receptor Antagonist Protein / Q000627:therapeutic use*; D008279:Magnetic Resonance Imaging; D008297:Male; D012871:Skin Diseases / Q000209:etiology*
chemical_list: D018501:Antirheumatic Agents; D053590:Interleukin 1 Receptor Antagonist Protein