Cerebral and Cutaneous Involvements of Xanthoma Disseminatum Successfully Treated with an Interleukin-1 Receptor Antagonist: A Case Report and Minireview

作者：Campero Mario^*; Campero Sebastian; Guerrero Julia; Aouba Achille; Castro Alex

来源：Dermatology, 2016, 232(2): 171-176.

DOI：10.1159/000442522

• Xanthoma disseminatum
• Central nervous system
• Non-Langerhans cell histiocytosis
• Contrast-enhancing brain lesions
• Diabetes insipidus
• Interleukin receptor antagonist

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.

• 出版日期2016