Amongst the latter group, Griscelli syndrome (GS) most closely resembles Vici syndrome, and is further subdivided in 3 clinically and genetically distinct groups (for review, [27]), of which only GS type 2 due to recessive mutations in RAB27A features prominent immunological involvement and hemophagocytic lymphohistiocytosis (HLH), whereas GS type 1 due to recessive MYO5A, the allelic Elejalde syndrome (ES) and GS type 3 due to recessive MLPH mutations only feature pigmentary abnormalities with or without primary neurological features, respectively, but not typically immunodeficiency. This evidence concerns the gene MYO5A and hemophagocytic syndrome.