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Acrokeratosis verruciformis - Skin
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Acrokeratosis verruciformis - Skin

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Contributors: Amy Fox MD, David Dasher MD, Jeffrey D. Bernhard MD, Noah Craft MD, PhD, Lindy P. Fox MD, Lowell A. Goldsmith MD, MPH, Michael D. Tharp MD
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Synopsis

Acrokeratosis verruciformis (AKV) is a rare autosomal dominant condition with sporadic cases also reported. Patients typically have closely grouped verrucous papules on dorsal hands and feet, elbows, and knees, occasionally with punctate keratosis of acral surfaces. Nail changes include striations, subungual hyperkeratosis, and distal nail V-shaped nicking. Men and women are equally affected.

Papules are often present at birth and increase in number in childhood and adolescence. Late presentation has been reported into the fifth decade.

Much of the published research on AKV has been spent exploring its relationship to Darier's disease, with early data demonstrating a common mutation in ATP2A2. There is some suggestion that AKV and Darier's disease are allelic.

For more information, see OMIM.

Codes

ICD10CM:
Q82.8 – Other specified congenital malformations of skin

SNOMEDCT:
400018004 – Acrokeratosis verruciformis of Darier disease

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Verrucae – These can be numerous in distribution, similar to AKV, but will lack associated acral hyperkeratosis, nail changes, and family history. Histology will show hyperplasia, acanthosis, and koilocytes.
  • Darier's disease – Thought to be allelic to AKV with classic clinical presentation of malodorous crusted papules coalescing into plaques in a seborrheic distribution. Acral Darier's disease with very similar nail changes and acral keratoses can be difficult to distinguish from AKV. Classic histology is suprabasal acantholysis.
  • Epidermodysplasia verruciformis – Rare autosomal recessive condition with diffuse verrucous lesions, which are at risk of developing squamous cell carcinoma due to human papillomavirus infection. Histology shows characteristic clear cell changes with small pyknotic nucleoli diffusely throughout epidermis. Verrucous papules will occur in locations beyond dorsal hands and feet, which can be beneficial in excluding this diagnosis.

Best Tests

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Therapy

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References

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Last Updated: 03/29/2017
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Acrokeratosis verruciformis - Skin
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Acrokeratosis verruciformis : Dorsum of foot, Dorsum of hand, Forearm, Knee, Anterior lower leg, Verrucous scaly papules
Clinical image of Acrokeratosis verruciformis
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