Other families with UFS lack HPSE2 mutations; some instead have biallelic variants of leucine-rich repeats and Ig-like domains 2 (LRIG2).13, 14 The most studied of the 3 mammalian Lrig proteins is Lrig1, a tumor suppressor that downregulates growth factor signaling.15, 16, 17 Belonging to the family of leucine-rich repeat–containing proteins, LRIG2 comprises multiple leucine-rich repeats, 3 Ig-like domains, a transmembrane segment, and a long cytosolic portion.18 This evidence concerns the gene LRIG2 and neoplasm.