(Erythermalgia)

Estimated incidence: 1.3/100.000. Paroxysmal acrosyndrome characterized by redness and edema of the extremities (hands or feet) accompanied by burning pains that is relieved by contact with the cold (cold water, ice). The apparent hyperemia is actually accompanied by tissue ischemia (partly cause of the pain) because there is a precapillary arteriovenous shunt.

The term of erythromelalgia is now reserved for unilateral or asymmetric forms accompanied by a low platelet count and which responds to the administration of aspirin.

There are three major causes:

-         primitive form [MIM 133 020]:  gain-of-function mutation of the SCN9A gene which codes for the voltage - dependent Nav. 1.7 sodium channel (chromosome 2q), which is expressed at the level of the sensory and sympathetic neurons

-         secondary forms in case of infection (Lyme disease, HIV), a myeloproliferative syndrome, systemic lupus erythematosus, ovarian or breast cancer, diabetes mellitus, certain drugs (calcium channel blockers)

-         probably autoimmune axonopathy (adolescence) that specifically affects the small nerve fibers.

Women are twice as often affected as men and 30 % of patients are less than 30 years old. Locoregional anesthesia and lidocaine patches seem effective.

Anesthetic implications: 

neuropathic pain, depression, avoid exposure to cold. Complex drug treatment. Locoregional anesthesia: epidural or peripheral blocks. Sub-hypnotic doses of propofol appear to be a useful adjuvant.

References : 

-         D’Angelo R, Cohen IT, Brandom BW. 
Continuous epidural infusion of bupivacaine and fentanyl for erythromelalgia in an adolescent.
Anesth Analg 1992; 74: 142-4.

-         Rauck RL, Naveira F, Speight KL, Smith BP. 
Refractory idiopathic erythromelalgia. 
Anesth Analg 1996; 82: 1097-101.

-         Harrisson CM, Goddard JM, Rittey CD. 
The use of regional anaesthetic blockade in a child with recurrent erythromelalgia. 
Arch Dis Child 2003; 88: 65-6

-         Paticoff J, Valoska A, Nedeljkovic SS, Oaklander AL. 
Defining a treatable cause of erythromelalgia: acute adolescent autoimmune small-fiber axonopathy. 
Anesth Analg 2007; 104: 438-41.

-         Cook-Norris RH, Tollefson MM, Cruz-Inigo AE, Sandroni P, Davis MPD, DaviS DMR. 
Pediatric erythromelalgia : a retrospective review of 32 cases at Mayo Clinic over a 37-year period. 
J Am Acad Dermatol 2011; 66:416-23.

-         Bouyahyaoui Y, Meziane M, Hanaae Z, Mikou O et al. 
Erythermalgie primitive familiale. A propos d’un cas. 
Arch Pédiatr 2013 ; 20 :369-71. Patel N, Chen E, Cucchiaro G. The complexity of pain management in patients with erythromelalgia. A&A Case Rep 2015 ; 5 : 151-3

-        Lorello GR, Perlas A.
Erythromelalgia and peripheral nerve block : a case report.
A&A Practice 2019 ; 12 : 264-6.

-        Ogawa S, Ueno H, Maruyama A, Amaya F.
Extensive lumbar sympathetic ganglion block combined with epidural block for primary erythromelagia : a case report.
A&A Practice 2020 ; 14 : e01325 

-        Zyani A, El Moutawakil El Alami M, Alkouh R, et al.
Severe pediatric erythromelalgia: a case report on multimodal pain management and the role of regional anesthesia.
Cureus 2024 ; 16(10): e71351. DOI 10.7759/cureus.71351

-        Parry MS, Brancolini SA, MD, Madeline Bireley M, Pickle AE, Cladis FP.
Utilization of propofol as an analgesic adjuvant in the multimodal management of acute Erythromelalgia secondary to Lyme disease neuropathy: a case report.
A&A Practice 2025;19:e01932

Updated: March 2025