Artículo de revista
Cerebral and Cutaneous Involvements of Xanthoma Disseminatum Successfully Treated with an Interleukin-1 Receptor Antagonist: A Case Report and Minireview
Fecha
2016Registro en:
Dermatology 2016;232:171–176
DOI: 10.1159/000442522
Autor
Campero Soffia, Mario
Campero, Sebastián
Guerrero Peralta, Julia
Aouba, Aouba
Castro, Alex
Institución
Resumen
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 difficultto-
treat non-Langerhans cell histiocytosis. Similar results
with anakinra have been observed in patients with Erdheim-
Chester disease and in multicentric reticulohistiocytosis.