Erythrokeratodermia with Ataxia, also known as Giroux- Barbeau syndrome, was first reported in 1972. In infancy and childhood, erythrokeratodermia with ataxia presents with groups of red, hardened, scaly skin plaques developing into a neurological syndrome in early adulthood consisting of impaired muscle coordination (ataxia), dysarthria (poorly articulated speech), decreased tendon reflexes, and involuntary, rhythmic movements of the eyes (nystagmus). In childhood the plaques usually disappear in the summer months. The plaques may completely disappear in young adulthood only to reappear later in life. Normally, the neurological deficits become apparent when these plaques reappear; in rare cases, however, neurological abnormalities may be present early in life.