History: Vohwinkel syndrome is a rare autosomal dominant disorder of keratinization, characterized by diffuse honeycomb pattern palmoplantar keratoderma, constricting bands of the digits with pseudo-ainhum, and starfish-shaped hyperkeratotic plaques on the dorsum of the hands and feet, elbows, and knees.  Two mutations of the epidermal differentiation complex (a missense mutation of the GJB2 gene coding connexin-26 on chromosome 13, and an insertional mutation of the loricrin gene on the epidermal differentiation complex on 1q21) have been described to be responsible for Vohwinkel syndrome.