Indian Journal of Dermatology
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CASE REPORT
Year : 2006  |  Volume : 51  |  Issue : 4  |  Page : 269-271

Griscelli syndrome


Department of Child Health Unit III, Christian Medical College, Vellore, India

Correspondence Address:
Prabhakar D Moses
Department of Child Health Unit III, Christian Medical College, Vellore - 632 004, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.30292

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Partial albinism with immunodeficiency is a rare and fatal immunologic disorder characterized by pigmentary dilution and variable cellular immunodeficiency. It was initially described in 1978. Primary abnormalities included silvery grayish sheen to the hair, large pigment agglomerations in hair shafts and an abundance of mature melanosomes in melanocytes, with reduced pigmentation of adjacent keratinocytes. We describe a child with Griscelli syndrome who presented with hepatitis, pancytopenia and silvery hair. The diagnosis was confirmed by microscopic skin and hair examination.


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