MVK and hyperimmunoglobulinemia D with periodic fever: Mevalonate kinase deficiency, also known as hyperimmunoglobulinemia D syndrome (OMIM 260920) is characterized by an autosomal recessive Mendelian inheritance pattern (5) and is allelic to another disorder, mevalonic aciduria (MA, OMIM 610377), characterized by a very low activity of the enzyme MVK.