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386 Although many different treatment regimens have been proposed, only a few are based on ev‐ idence from randomized controlled trials. Only vemurafenib was approved by the Food and Drug Administration in November 2017 for patients with BRAFV600E ‐mutant ECD. The treatment reg‐ imens proposed in the literature include corti‐ coids, interferon alfa, anakinra, infliximab, tocili‐ zumab, cyclophosphamide, anthracyclines, vinca alkaloids, vemurafenib, dabrafenib, and a com‐ bination therapy of dabrafenib and trametinib. A large case series has been published that sup‐ ports the interferon alfa treatment.4 The efficacy of interferon α differs among pa‐ tients, depending on the site of disease involve‐ ment. In addition, interferon alfa has little or no impact in cases of severe disease, multiorgan disease, or specific organ involvement including the central nervous system as well as pulmonary and cardiovascular systems.5

Significant response to dabrafenib in a patient with Erdheim–Chester disease with BRAFV600E mutation