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Griscelli syndrome type-3
Bela J Shah1, Ashish K Jagati1, Nilesh K Katrodiya1
1Department of Dermatology, BJ Medical College and Civil Hospital, Ahmedabad, Gujarat, India.
Abstract:
Griscelli syndrome (GS) is a rare autosomal recessive multisystem disorder of pigmentary dilution of skin, silver gray hair, variable immunodeficiency, neurological impairment, and abnormal accumulation of melanosomes in melanocytes. GS type 3 is characterized by hypomelanosis with no immunological and neurological manifestation. Prognosis is very good in type 3 GS and usually require no active intervention, as opposed to type 1 and 2 where early diagnosis and treatment plays a crucial role in patient's survival. The characteristic phenotypic appearance, especially the pigment dilution of the patient's hair, is emphasized here.
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