RESUMO
INTRODUCTION: Primary erythermalgia is a rare acrosyndrome due to paroxystic arteriolar dilatation of extremities that become red and congested 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 be effective in mother and 14-year-old child who had never drawn benefit from any previous treatment. DISCUSSION: Several etiologies may be responsible for erythermalgia according to the clinical type: primary erythermalgia with pediatric onset, and secondary erythermalgia as a reaction to myeloproliferation, other diseases, drug or heavy metal poisoning. Pathophysiology of primary erythermalgia is not clearly understood and the main concern is therapy: in this respect, pizotifene deserves to be considered as a valuable possibility.