Transgrediens et progrediens palmoplantar keratoderma (Greither's disease)with particular histopathologic findings

We describe a patient with transgrediens et progrediens palmoplantar kerato derma (Greither's disease). Ten of the 25 members of this patient's family in six consecutive generations were affected by the disorder. The pedigree was consistent with an autosomal dominant inheritance pattern, With variabl e penetrance. The proband's physical examination showed typical signs of Gr either's disease. The most striking findings were seen in histopathologic s tudy and consisted of round, focal areas of orthohyperkeratosis located on delled areas of the epidermis. These histopathologic features were present in both the lesions of the palms and dorsum of the hands. We review the cli nical manifestations and histopathologic findings of this particular varian t of palmoplantar keratoderma and the differential diagnosis.