The family of palmoplantar keratodermas are relatively uncommon diseases. The clinical manifestations are varied and exuberant, attracting the dermatologists' attention. Although the majority are limited to the skin, some present systemic clinical repercussions, above all in the ectodermal derivatives. Due to the clinical variability, as well as to the poorly understood etiopathogenic mechanisms, several classifications have been proposed. However, none has attained total universal acceptance, such that disagreement is common between the various authors with regard not only to the classifications, but also regarding the existence of some types of keratoderma that are sometimes considered as variants, while other times as new entities. Recently, better understanding of the structure and dynamics of the epidermis, especially the cellular cytoskeleton, intercellular adhesion system and ultra-structure of the basal membrane, has enabled a concrete clarification of the origin of such processes. The cytoskeleton and above all the cytokeratins, have been the frequent subject of studies and identified as responsible for many of the palmoplantar keratodermas. The present case refers to a patient with a non-transgressing diffuse palmoplantar keratoderma, with onset in childhood and several familial cases. The clinical-histopathological evaluation enabled the diagnosis of Vörner type epidermolytic hyperkeratosis. Such a case report is justified by the rarity and exuberance of the picture, with association of urothelial cancer in the same patient, and it underscores the importance of the histopathological evaluation for the correct diagnosis of keratodermas.
keratoderma; palmoplantar; keratin
