The authors report on a case of chronic urticarial eruption since infancy in a man who is now 27-year-old; the association of numerous chronic symptoms, principally meningo-encephalitis, polyarthritis, hepato-spleno-adenomegaly allows the authors to compare this case to those described by Prieur and Griscelli in 1981 and 16 others cases found in literature. It appears as a "systemic disease" without biological or immunological markers; it might belong to the group of chronic normocomplementemic vasculitis.