ABSTRACT
Oculocutaneous albinism represents a group of genetically determined diseases that affect a considerable population. It was recognized by Garrod [1] in 1908 as an inborn error of metabolism in which he postulated a defect in an intracellular enzyme of melanin synthesis. Albinism is found in fish, birds and mammals, including all human races. The primary defect maybe restricted to the pigment cell (melanocyte) system or may involve many cell types, among them the melanocytes. The melanocytes are specialized cells in which the biosynthesis of melanin occurs in the melanosomes. The cells arise from melanoblasts that develop early in embryogenesis in the neural crest and the neuroectoderm. Melanocytes migrate to the choroid, iris and retina and to the skin where melanosomes are transported to keratinocytes and hair producing pigment.
