Abstract
ALPK1 somatic mutation (p.V1092A) during fetal development can cause spiradenoma or cylindroma to occur early in life along a line of Blaschko. Excision using Mohs micrographic surgery can delineate the subclinical extent and may reduce future risk of spiradenocarcinoma where ALPK1 is a driver mutation. Our case supports the testing of CYLD and ALPK1 in skin tissue of suspected mosaic cases of CYLD cutaneous syndrome.
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