SILVERY HAIR WITH IMMUNODEFICIENCY: A COMPARATIVE CLINICAL BRIEF OF GRISCELLI SYNDROME TYPE II AND CHEDIAK HIGASHI SYNDROME.

One of the rare but a common symptom of some genetic diseases presenting in Pediatric age group is silvery hair. These disorders termed as "silvery hair syndrome" consist of Griscelli syndrome (GS), Chediak-Higashi syndrome (CHS) and Elejalde disease. Among these syndromes primary immunodeficiency is present only in CHS and GS type II. CHS which is characterized by severe defect in phagocytic function leading to recurrent infections with pyogenic organism, silver blond hair, partial oculocutaneous albinism, progressive motor or sensory neurologic defects and bleeding tendencies is a rare autosomal recessive defect. Griscelli syndrome 2 is also a rare disorder with autosomal recessive inheritance associated with dilution of pigments in skin, hair, splenohepatomegaly, immune and neurological dysfunction and pancytopenia. Because of overlapping clinical presentation, they closely mimic each other. Hence here we are focusing on differentiating these two disorders and highlighting the diagnostic dilemma. We report two cases which were referred to our tertiary care center of central India with varied clinical manifestations and complications, sharing a common feature of silvery hair. [ABSTRACT FROM AUTHOR]