Family primary Érythromelalgie

Described for the first time in 1878 the family primary érythromelalgie appears by pains important, recurring, bilateral and symmetrical being accompanied by heat, redness and swelling on the level of the feet and much less frequently of the hands. These demonstrations are strictly isolated and there does not exist any other implied body; their intensities and their durations vary in the same family.

This disease appears in great childhood or in adolescence but can also appear earlier. Located at the beginning with the plant of the feet and the hands, it can reach later the legs and the front armlevers with pains several times per day.

At the beginning, the pains are started by an exposure to heat but by the effort, of the tightened shoes, the wearing of sock, alcohol and the food spiced. Some people complain about allodynie (stimulation usually not causing a pain) or about hyperalgesy (excessive sensitivity to the pain).

This pathology can involve sleep disorders and limit in an important way the daily activities. The Biopsie of skin shows nonspecific contractings of the basal Membrane of the vessels with an edema, mononuclear infiltrations of cells and a reduction in the innervation by the autonomous nervous system.

There does not exist specific treatment of this disease.

Sources

Fuki Mr. Hisama, Sulayman D Dib-Hajj, Stephen G Waxman, SCN9A-Related Inherited Erythromelalgia In: GeneReviews At GeneTests: Medical Genetics Information Resource (database online). Copyright, University off Washington, Seattle. 1997-2006. ===Références===

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