Introduction. Primary erythermalgia is a rare acrosyndrome due to paro
xystic arteriolar dilatation of extremities that become red and conges
ted with warmth and burning sensation or pain. Observation. We report
herein the observation of a primary erythermalgia affecting 3 degrees
in the same family (grandfather, uncle/mother, and children with early
onset at 6 years): pizotifene, an antiserotoninergic drug proved to b
e effective in mother and 14-year-old child who had never drawn benefi
t from any previous treatment. Discussion. Several etiologies may be r
esponsible for erythermalgia according to the clinical type: primary e
rythermalgia with pediatric onset, and secondary erythermalgia as a re
action to myeloproliferation, other diseases, drug or heavy metal pois
oning. Pathophysiology of primary eryrhermalgia is not clearly underst
ood and the main concern is therapy: in this respect, pizotifene deser
ves to be considered as a valuable possibility.