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: Mario Campero, Sebastián Campero, Julia Guerrero, Achille Aouba, Alex Castro
• Journal: Dermatology (Basel, Switzerland) (Dermatology) Vol. 232 Issue 2 Pg. 171-6 ( 2016) ISSN: 1421-9832 [Electronic] Switzerland
• PMID: 26741816 (Publication Type: Case Reports, Journal Article, Review)
• Copyright: © 2016 S. Karger AG, Basel.

Chemical References

• Antirheumatic Agents
• Interleukin 1 Receptor Antagonist Protein

Topics

• Adult
• Antirheumatic Agents (therapeutic use)
• Epilepsy, Temporal Lobe (diagnostic imaging, etiology)
• Histiocytosis, Non-Langerhans-Cell (complications, drug therapy)
• Humans
• Hypopituitarism (diagnostic imaging, etiology)
• Interleukin 1 Receptor Antagonist Protein (therapeutic use)
• Magnetic Resonance Imaging
• Male
• Skin Diseases (etiology)