Vohwinkel Syndrome Clinical Presentation

Updated: Aug 13, 2019
  • Author: Zoltan Trizna, MD, PhD; Chief Editor: Dirk M Elston, MD  more...
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Presentation

History

The classic skin lesions develop at an early age. Explore the family history for abnormalities suggestive of Vohwinkel syndrome.

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Physical Examination

The classic triad is as follows:

  • Diffuse palmoplantar keratoderma in a honeycombed pattern

  • Constricting bands of the digits with autoamputation (pseudoainhum)

  • Starfish-shaped hyperkeratotic plaques on the dorsum of the hands and feet, elbows, and knees (In addition, linear hyperkeratoses may be observed on the elbows and knees.)

Note the images below:

Starfish-shaped plaques. Starfish-shaped plaques.
Palmar hyperkeratosis. Palmar hyperkeratosis.
Pseudoainhum. Pseudoainhum.

Rare associated findings include scarring alopecia and nonprogressive sensorineural hearing loss at high frequencies (classic variant).

A case report of two siblings described a probable new variant of Vohwinkel syndrome with congenital hypotrichosis. [10] Cases with mental retardation [11] and an ichthyosiform presentation [12] have also been described.

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Complications

Complications can include loss of digit(s) with functional impairment of the limb and hearing loss.

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