[Muckle-Wells syndrome or association of joint pain attacks, urticarial outbreaks and sensory deafness?]

The Muckle and Well's syndrome corresponding to a transmission of the autosomic dominant type, combines bouts of urticaria, episodes of arthralgias to a shrinking of the ear and a sensory deafness. Sometimes, it evolves into a renal amylosis. Sometimes, as the case presented here, it combines multiple malformations. Its place in nosology is imprecise. It is, at the same time, close to systemic urticaria, sensory deafness, amylosis and specially amylosis of the periodic disease. But the common link between the various elements of the syndrome remains undetermined, for the time being.