Griscelli syndrome is a rare autosomal recessive[1] disorder characterized by albinism (hypopigmentation) with immunodeficiency, that usually causes death by early childhood.

Type 1 Griscelli syndrome is associated with severe brain function issues along with distinctive discolouring of the hair and skin.

Type 2 Griscelli syndrome have immune system abnormalities in addition to hypopigmentation of skin and hair.

Under light microscopy, these hairs exhibit bigger and irregular melanin granules, distributed mainly near the medulla.

People with Griscelli syndrome type 1 have severe problems concerning brain function, typically characterized as delayed development, intellectual disability, seizures, hypotonia, and eye and vision abnormalities.

People with Griscelli syndrome type 2 have immune system abnormalities and are susceptible to recurrent infections; they develop hemophagocytic lymphohistiocytosis, which may damage organs and body tissues.

People with Griscelli syndrome type 3 only have the baseline light skin and hair coloring abnormalities.

[7] In order to treat patients, the aggressive therapy strategy approach must always be taken for acute bacterial infections and prophylactic antibiotics.