The first condition is a Proteus-like syndrome with a distinct type of epidermal nevus (also referred to as segmental overgrowth, lipomatosis, arteriovenous malformation, and epidermal nevus (SOLAMEN) syndrome or type 2 segmental Cowden syndrome) that likely relates to the early LOH at the PTEN allele in the affected tissues in patients with underlying germline PTEN mutation [96,97,98]. Here, PTEN is linked to Proteus-like syndrome.