The disease is characterized by the darkening of the urine in contact with air, due to the lack of an enzyme.
Occurs an accumulation of pigment or ochronosis, which is going to damage the connective tissue ( bones, joints, cartilage, muscle, etc) The pain is the biggest problem of this disease, which usually begins in the spine, at the level of the lumbar area.
With the years can occur deposit of pigment both in the sclera as in the pabellos of the ear, in the cartilage, there is a stain dark