A Griscelli Syndrome, with Retropharyngeal Abscess, as First Clinical

Griscelli syndrome (GS) is a rare autosomal recessive disorder, which is characterized by albinism with immunodeficiency and usually causes death in early childhood. Accordingly, this syndrome is a primary immune defects presented with a dilution of pigmentations of the skin and hair, recurrent pulmonary and skin infections, neurologic disorders, hypogammaglobulinemia, and variable cellular immunodeficiency. Moreover, in different phenotypes of the syndrome, three mutations have been mentioned. In most of them, GS leads to death in the first decade of life. Herein, we reported a one-year- old male child with an upper respiratory infection and retropharyngeal abscess as the first clinical manifestation.