Abstract

Chediak-Higashi syndrome, Griscelli syndrome (GS) and Elejalde disease are rare autosomal recessive disorders known as silvery hair syndromes. Here, we describe a case of a 7-year-old girl with bronze skin and silvery hair, diagnosed as GS. Three genetic types of GS are known. Pigmentary dilution is common to all three and is the only finding in Griscelli Type 3. Type 1 is associated with neurological problems and Type 2 with immunodeficiency.

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