Biallelic LOF mutations in the gene encoding for the cell membrane-cytoskeleton-associated protein RLTPR (RGD, leucine-rich repeat, tropomodulin and proline-rich-containing protein), also known as CARMIL2 (capping protein regulator and myosin 1 linker 2), have been shown to be responsible for a novel PID disorder characterized by cutaneous and pulmonary allergy, by various bacterial, fungal, and mycobacterial infections, as well as by EBV lymphoproliferation (EBV+ smooth muscle tumors) (127, 128). The gene discussed is CARMIL2; the disease is smooth muscle tumor.