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The Hiper-IgD Syndrome (HIDS) is an autoinflammatory disease characterized by recurrent febrile episodes each 4–8 weeks accompanied by an intense inflammatory reaction, lymphadenopathy, abdominal pain, diarrhea, arthralgias, hepatosplenomegaly and cutaneous signs [1]. Mutations in the gene that codifies the enzyme mevalonate kinase (MVK), located in the chromosome 12q24, have been demonstrated to be the cause of this syndrome [2].

Hiper IgD syndrome (HIDS): clinical and genetic features in five patients