• Presence of BRAF mutationBRAF inhibitor (vemurafenib or dabrafenib)± systemic corticosteroids, if oedema or acute symptoms• Absence of BRAF mutationIf cardiac/neurologic disease or end-organ dysfunction:MEK inhibitor (cobimetinib)If low-burden disease involving bones and retroperitoneum:Cytokine-directed therapy (anakinra)± systemic corticosteroids, if oedema or acute symptoms• No access to BRAF or MEK inhibitorsIf high-burden disease:IFN-a/PEG–IFN-aor Cladribine (rare)± systemic corticosteroids, if oedema or acute symptoms. This evidence concerns the gene BRAF and nervous system disorder.