Keratoderma hereditaria mutilans (vohwinkel′s syndrome)

We report two cases of Vohwinkel′s syndrome in son and mother presenting with a progressive palmoplantar keratoderma with ′honeycomb′ appearance associated with constricting bands encircling digits of hands and feet along with keratotic plaques on dorsum of hands and feet. Both had associated sensorineural deafness.