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December 1962

Acrokeratosis Verruciformis (Hopf): A Follow-Up Study

Author Affiliations


Professor of Dermatology, Temple University School of Medicine (Dr. Niedelman); Professor of Medicine, Division of Medical Genetics, Department of Medicine, Johns Hopkins University School of Medicine (Dr. McKusick).

Arch Dermatol. 1962;86(6):779-782. doi:10.1001/archderm.1962.01590120077013

In 1947 Niedelman1 described an Italian-American family in which acrokeratosis verruciformis (Hopf) occurred in 14 members in 4 generations. The inheritance followed a dominant pattern. Van den Bosch2 has recently suggested that the condition is an X-linked dominant rather than an autosomal dominant. The suggestion was based on the facts that females are about twice as frequently affected as males and that no certain instance of male-to-male transmission has been reported. Assuming complete penetrance, the condition if X-linked, should, furthermore, affect all daughters of an affected male. Van den Bosch was prompted to analyze the reported pedigrees by his own observations3 of a complex, apparently X-linked trait in which acrokeratosis verruciformis is one component.

Follow-up of the family reported in 1947 seemed indicated, in order to corroborate the