Epidermolytic palmoplantar keratoderma

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Synonyms: Diffuse erythrodermic palmoplantar keratoderma, Voerner type | Diffuse erythrodermic palmoplantar keratoderma, Vörner type | EPPK | Epidermolytic palmoplantar keratoderma of Voerner | Epidermolytic palmoplantar keratoderma of Vörner

A rare non-syndromic hereditary palmoplantar keratoderma characterized by diffuse yellowish thick hyperkeratosis of the palms and soles with a sharp demarcation at the volar border and an erythematous margin and the epidermolytic pattern of changes on the skin biopsy including perinuclear vacuolization granular degeneration of keratinocytes in the spinous and granular layer and tonofilament aggregates. Painful fissures and hyperhidrosis are frequently associated.

Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview. Reference: Access aggregated data from Orphanet at Orphadata. Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http://www.orphadata.org. Data version June 2024

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