Erythromelalgie primitive, une nouvelle observation

Erythromelalgia is a vascular acrosyndrome first described by Mitchell in 1878. It is rare, affecting the feet more than the hands, and the symptoms are triggered by heat and calmed by cold. There are two types: the primary form (exceptional hereditary form and non-familial idiopathic form), the secondary form (associated with hematological disorders and systemic diseases). The exact mechanism is the subject of controversy. It has an important functional impact. We report a new observation. It was a 39-year-old woman, multiparous, in a polygamous marriage, residing in Gao, referred to the emergency room on February 12, 2018 for severe hand pain. She had presented with the same episode in March 2011 relieved by hand immersion in cold water associated with an unidentified analgesic. No similar conditions were found in her family. On examination, the general condition was good, we noted a large placard of both hands, red with a salmon appearance, taking the eminences in a V shape, sparing the back of the hands. The lesion was warm and painful to the touch, without any portal of entry (A, B, C). Blood count, sedimentation rate, blood glucose and antinuclear antibodies were normal. 500mg/day acetylsalicylic acid treatment was instituted. The evolution under this treatment was favorable with complete remission after two weeks (D). The diagnosis of primary idiopathic erythromelalgia was evoked in view of the patient's age, the clinical and biological signs and the response to treatment.