ADAM17 deficiency is considered an autoinflammatory disease (6) caused by autosomal recessive mutations in the ADAM17 gene, encoding TNF-α converting enzyme (TACE), which is necessary for the cleavage and secretion of TNF-α, epidermal growth factor, transforming growth factor alpha (TGF-α), and some desmogleins (6, 133). The gene discussed is ADAM17; the disease is hyperinsulinemic hypoglycemia, familial, 4.