Silvery gray hair syndromes: An insight into diagnosis

L Hemalata, R Pradeep, Supriya Siddavatam, Asha Benakappa


Context: Silvery gray hair syndromes consist of three conditions, Chediak–Higashi syndrome (CHS), Griscelli syndrome (GS), and Elejalde syndrome (ES), which are rare autosomal recessive diseases presenting in childhood. They have similar skin and hair changes but differ with respect to the other clinical findings. Aim: This retrospective study was taken up to identify the
spectrum of silvery gray hair syndromes diagnosed over a period of 10 years (2006-2015) in a super specialty pediatric institute and also to identify characteristic clinical and diagnostic findings. Methods: A total of 17 children diagnosed to have silvery gray hair syndrome over a period of 10 years (2006-2015) at Indira Gandhi Institute of Child Health Hospital, Bengaluru and
were studied retrospectively. Complete blood counts, peripheral smear study, and microscopic examination findings of the hair were analyzed. Radiological findings were also considered. Results: The most common syndrome in the study was GS Type II (10 cases) followed by CHS (5 cases), with one case each of GS Type III and ES. Conclusion: This study emphasizes the
importance of silvery gray hair as a clue to diagnose these rare genetic disorders with proper clinical examination and simple diagnostic procedures.


Autosomal recessive, Chediak–Higashi syndrome, Elejalde syndrome, Griscelli syndrome

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Copyright (c) 2017 Hemalata L, Pradeep R, Supriya Siddavatam, Asha Benakappa

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