Therapeutics
Agam B. Jagota, DO
Resident Physician
UTHealth Houston
Houston, Texas, United States
Garrett H. Hayes, MPH
Medical Student
UT Southwestern Medical Center
Plano, Texas, United States
Gregory Blazek, MD
Resident Physician
University of Texas Health Science Center at Houston
Houston, Texas, United States
Brian Bruel, MD
Attending Physician, Adjunct Professor
UTHealth Houston
Houston, Texas, United States
Erythromelalgia is a neurovascular pain syndrome characterized by recurrent and severe episodes of burning pain and erythema that is commonly triggered by heat and relieved with cooling. The mechanism of primary erythromelalgia is due to a gain of function mutation in the SCNA9 gene which encodes the NaV1.7 voltage-gated sodium channel which is highly-expressed in sympathetic ganglion neurons and dorsal root ganglions. The gain of function mutation in these channels lowers the threshold for activation and increases pain signaling in response to heat. Suzetrigine (Journavx) is a selective inhibitor of the NaV1.8 voltage-gated sodium channel, which is expressed in peripheral sensory neurons that transmit pain signals to the spinal cord and brain. Currently, suzetrigine’s only FDA approved indication is for the treatment of moderate to severe acute pain in adults.
Conclusions: While on Suzetrigine, he did not experience flares of his erythromelalgia, suggesting NaV1.8 inhibition as a strategy for sustained relief from erythromelalgia symptoms.