Acrokeratosis verruciformis of Hopf

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Acrokeratosis verruciformis of Hopf

Synonyms: AKV of Hopf

A rare genetic acrokeratoderma disease characterized by multiple symmetrical asymptomatic skin-colored (rarely brownish) flat-topped wart-like papules located on the dorsal aspects of the hands and feet (occasionally found on other parts of the body such as knees elbows and forearms) typically associated with palmoplantar punctate keratosis and variable nail involvement (including leukonychia thickening ridging longitudinal striations and splitting). Histology reveals undulating hyperkeratosis papillomatosis hypergranulosis and acanthosis creating a characteristic ‘church spire’ appearance with no acantholysis nor dyskeratosis 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: // Data version October 2023.

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