摘要IntroductionMutilating palmoplantar keratoderma (MPK), also known as Vohwinkel syndrome (VS), is a rare autosomal dominant genodermatosis. It is manifested by diffuse honeycomb-like keratotic thickening in the skin of the palms and soles, starfish-shaped keratotic plaques on the dorsum of the hands and feet, and fibrous constricting bands (pseudoainhum) at the interphalangeal joints of the hands and feet, which may compromise neurovascular function and mobility and even result in autoamputation.1 This condition usually occurs on the fifth digits; it often manifests in early childhood but becomes more evident in adulthood. Some features have been noted to be associated with MPK, such as alopecia, hearing loss, spastic paraplegia, myopathy, ichthyosiform dermatoses, and nail abnormalities.