Successful treatment of cutaneous-onset Erdheim–Chester disease with cobimetinib and anakinra

Author:

Aromolo Italo Francesco1ORCID,Moltrasio Chiara23,Maronese Carlo Alberto1ORCID,Campochiaro Corrado4ORCID,Bonometti Arturo5,Berti Emilio1,Passoni Emanuela2,Marzano Angelo12

Affiliation:

1. Department of Pathophysiology and Transplantation, University of Milan , Milan , Italy

2. Dermatology Unit, IRCCS Foundation Maggiore Policlinico Hospital , Lombardia, Milan , Italy

3. Department of Medical Surgical and Health Sciences, University of Trieste , Trieste, Friuli-Venezia Giulia , Italy

4. San Raffaele Hospital , Milan , Italy

5. Pathology Unit, Humanitas University , Rozzano, Milan , Italy

Abstract

Erdheim–Chester disease is a rare multisystem non-Langerhans cell histiocytosis. We report the case of a patient with BRAF wildtype Erdheim–Chester disease presenting with facial nodular xanthomas and developing systemic involvement 10 years later. The patient was successfully treated with a combination of anakinra and cobimetinib.

Publisher

Oxford University Press (OUP)

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