Slivers of Hair - A Clue to Uncover Silver Hair Syndromes

Silver hair syndromes (SHS) consist of rare autosomal recessive disorders, Chediak-Higashi syndrome (CHS), Griscelli syndrome (GS), and Elejalde disease. CHS is a disorder of vesicle trafficking; characterized by silvery hair, diffuse pigment dilution, immunodeficiency, bleeding diathesis, neurological manifestations, and an accelerated phase due to lymphohistiocytic infiltration. GS is characterized by hypopigmentation of skin and hair, with large clumps of pigment in the hair shaft. There are three types of GS. GS1 and GS2 show neurologic and hematologic impairments; GS3 is restricted to the skin. Some authors suggest that Elejalde syndrome is synonymous with GS Type 1. Here, we report two cases who presented with silver-gray hair but with varied clinical manifestations. With a light microscopic examination of the hair and peripheral smear, a diagnosis has been made. This report highlights the importance of hair shaft microscopy, an inexpensive, noninvasive, and simple tool in the diagnosis of SHS.