DOCK11 and autoinflammatory disease, multisystem, with immune dysregulation, X-linked: X-linked multisystem autoinflammatory disease with immune dysregulation (ADMIDX) is caused by hemizygous mutations in the DOCK11 gene, located on chromosome Xq24 [1]. DOCK11 is primarily expressed in hematopoietic cells, and its guanine nucleotide exchange factor (GEF) activity modulates actin cytoskeleton remodeling through activation of the small Rho GTPase CDC42 [2,3]. Germline hemizygous loss-of-function mutations in DOCK11 result in a primary immunodeficiency due to impaired hematopoiesis and immune function [2].