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Image Number #5584 (Griscelli syndrome)

Diagnosis: Griscelli syndrome

Description: Partial albinism with recurrent skin infections.

Morphology: Hair white

Site: Face

Sex: F

Age: 3

Type: Clinical

Submitted By: Nameer Al-Sudany

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Differential Diagnosis
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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.

DermNetNZ   eMedicine   PubMed   Dermatology Online   Archives   JAAD for "Griscelli syndrome"

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