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

Mario Campero; Sebastián Campero; Julia Guerrero; Achille Aouba; CASTRO MENDEZ, ALEX

doi:10.1159/000442522

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

Journal

Dermatology

ISSN

1018-8665

1421-9832

Date Issued

2016

Author(s)

Mario Campero

Sebastián Campero

Julia Guerrero

Achille Aouba

CASTRO MENDEZ, ALEX

Facultad de Medicina Clínica Alemana Universidad del Desarrollo

Type

Resource Types::text::journal::journal article

DOI

10.1159/000442522

URL

https://investigadores.udd.cl/handle/123456789/7427

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.