The characteristic clinical features include sparse to absent scalp hair, sparse eyebrows, and eyelashes, abnormal dentition (peg-shaped, conical crowns and enamel defects), hypoplastic nails, palmoplantar keratoderma and bilateral partial cutaneous syndactyly variably affecting the fingers and toes.3, 4 EDSS1 has been reported to be caused by variants in the Poliovirus Receptor Related-4 (PVRL4) gene recently named Nectin Cell Adhesion Molecule-4 (NECTIN4). Here, NECTIN4 is linked to Palmoplantar keratoderma.