albinism

—albinistic, adj.

/al"beuh niz'euhm/, n.

the state or condition of being an albino.

[1830-40; ALBIN(O) + -ISM]

* * *

Absence of the pigment melanin in the eyes, skin, hair, scales, or feathers.

It arises from a genetic defect and occurs in humans and other vertebrates. Because they lack the pigments that normally provide protective coloration and screen against the sun's ultraviolet rays, albino animals rarely survive in the wild. Humans have long intentionally bred certain albino animals (e.g., rabbits) for their appearance. In humans with generalized, or total, albinism, the affected person has milk-white skin and hair; the iris of the eye appears pink, the pupil red. Vision abnormalities such as astigmatism, nystagmus (rapid involuntary oscillation of the eye), and photophobia (extreme sensitivity to light) are common. Generalized albinism occurs throughout the world in about one in 20,000 persons.

* * *

▪ genetic condition

      (from the Latin albus, meaning “white”), the absence of pigment in the eyes, skin, hair, scales, or feathers. Albino animals rarely survive in the wild because they lack the pigments that normally provide protective coloration and screen against the sun's ultraviolet rays.

      Albinism is a hereditary condition in humans and other vertebrates. In generalized, or total albinism, the affected person has milk-white skin and hair, though the skin may be slightly pinkish in colour owing to underlying blood vessels. The iris of the eye appears pink, while the pupil itself appears red from light reflected by blood in the unpigmented choroid. Vision abnormalities such as astigmatism, nystagmus (rapid involuntary oscillation of the eye), and photophobia (extreme sensitivity to light) are common. Albinos sunburn very easily and are thus more susceptible to skin cancers.

      Generalized albinism is caused by the absence of melanin, the dark brown pigment normally present in the human skin, hair, and eyes. Albinism arises from a genetic defect resulting in the body's inability to synthesize tyrosinase, an enzyme that is necessary for the production of melanin. When the gene for the production of tyrosinase is absent and the gene for no production of the enzyme is present in a double form, albinism will result. Human albinism is transmitted as an autosomal recessive trait. Generalized albinism occurs in all races in about one in 20,000 persons.

      A related disease is vitiligo, in which localized areas of the skin lack pigment and resemble that of an albino, while elsewhere on the body the pigmentation is normal.

* * *