Pilar Sheath Acanthoma, also known as acanthoma, pilar sheath, is related to acanthoma and ataxia with vitamin e deficiency. An important gene associated with Pilar Sheath Acanthoma is CLEC2A (C-Type Lectin Domain Family 2 Member A), and among its related pathways/superpathways are Pre-NOTCH Expression and Processing and Development Notch Signaling Pathway.