Light and scanning electron microscopic examination of hair in Griscelli syndrome

Abstract

Griscelli syndrome is a rare disease characterized by pigment dilution, partial albinism, variable cellular immunodeficiency, and an acute phase of uncontrolled T-lymphocyte, and macrophage activation. Griscelli et al described this syndrome in 1978. Since then, only in approximately 60 cases have been reported, most from the Turkish, and Mediterranean population. In microscopic examination, silvery gray hair with large, clumped melanosomes on the hair shaft is the diagnostic finding. Here, we present scanning electron microscopic study of hair in 2 cases of Griscelli syndrome, where the hair showed normal cuticular pattern but nodular structures were present as an abnormal findings.