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Image Number 2512 (Griscelli syndrome)

 
Morphology:
Hair white

Diagnosis: Griscelli syndrome

Site: Face

Sex: F

Age: 3

Type:

Description: Partial albinism with recurrent skin infections.

Submitted by: Nameer Al-Sudany

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Differential Diagnosis

History:

A 3-year-old female toddler suffered recurrent skin infections (pyogenic pyodermas, bolis and impetigo) for more than one year duration. Partial albinism with gray-silvery white hair have been noticed since birth. On investigations, neutropenia, thrombocytopenia and hypogammaglobulinemia was detected. Direct hair examination under light microscope showed unevenly distributed melanin aggregates in the medulla.

N.B: Thewhite color of the scalp hair was masked by henna (hair dye of plant origin) at time of presentation.



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