Acrokeratosis Verruciformis of Hopf Clinical Presentation

Updated: May 11, 2018
  • Author: Sarah Sweeney Pinney, MD; Chief Editor: William D James, MD  more...
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Presentation

History

Acrokeratosis verruciformis is usually present at birth or manifests in early childhood, but the onset may be delayed until the fifth decade of life. Acrokeratosis verruciformis of Hopf has an autosomal dominant mode of transmission, but sporadic cases also occur. [15, 21]

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Physical Examination

Dry, rough, skin-colored or reddish-brown, flat-topped, or warty papules resembling flat warts are observed, particularly on the dorsum of the hands and, at times, on the dorsum of the feet, as shown in the image below.

Papules also may be found on the knees, elbows, forearms, or lower legs. Small groups or isolated papules may develop on other parts of the body. Papules sometimes are more easily felt than seen.

Palmar skin may be thickened and may show punctate keratosis, pits, or punctiform breaks in dermatoglyphics, identical to those observed in persons with Darier disease or Grover disease. [22]

Nail involvement, including longitudinal splitting, striations, and subungual hyperkeratosis, also may be seen. [23]

Variants can also present on the trunk, as is seen in a case following the lines of Blaschko. [24]

This photos shows flat-topped, hyperkeratotic, ski This photos shows flat-topped, hyperkeratotic, skin colored papules on the dorsal hands without palmar involvement. This patient also has dystrophy of the fingernails, which is not easily visible in this photograph. Biopsy demonstrated epidermal acanthosis and papillomatosis with hyperkeratosis. Photo courtesy of Sarah S. Pinney, MD.
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