Vohwinkel syndrome (VWS) is a rare autosomal dominant inherited palmoplantar keratoderma (PPK) caused by a mutation in the GJB2 gene that encodes connexin 26. Connexin 26 is found in gap junctions in the skin and the cochlea. It plays a role in skin and inner ear homeostasis, as gap junctions function in allowing flow of nutrients and signaling molecules between cells.

The syndrome typically presents in infancy or early childhood. The PPK classically shows a diffuse honeycomb pattern. Additional features include starfish-shaped keratotic plaques on dorsal hands, feet, elbows, and knees as well as constricting digital bands around fingers and toes termed pseudoainhum, which may progress to autoamputation. High-frequency hearing loss is typically associated.