(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]: 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 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 aged less than 30 years. The rare cases reported in the literature show that the use of a regional anesthesia technique has usually little effect and does not reduce the doses of complex medical treatment. Lidocaine patches seem effective.

Anesthetic implications: 

neuropathic pain, depression, avoid exposure to cold.

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

Updated: February 2021