ACROKERATOSIS VERRUCIFORMIS OF HOPF
By Gary M. White, MD
Acrokeratosis verruciformis of Hopf (AKV) is a benign, dominantly inherited presence of keratotic papules on the dorsal hands and feet.
- Onset is usually at birth or during early childhood, but the onset may be delayed up to the fifth decade.
- The cause is a heterozygous missense mutation in ATP2A2 gene, the gene involved in Darier disease (DD).
Acrokeratosis verruciformis of Hopf vs Darier Disease
- AKV and DD have overlapping clinical features.
- Both diseases result from mutations in the same ATP2A2 gene.
- Identical mutations in ATP2A2 in AKV and DD have not been reported to date [Am J Dermatopathol. 2012 Aug;34(6):597-601]
- Their histopathological features are different.
Flesh-colored, hyperkeratotic papules on the dorsa of the hands and feet is characteristic. In some cases, they may be widespread, affecting the knees, elbows, forearms, or lower legs. Palmar pits and/or keratoses may be seen. Nail involvement may occur in the form of longitudinal splitting, striations, and subungual hyperkeratosis.
Acrokeratosis verruciformis of Hopf on the dorsal hands may look identical to stucco keratoses. e.g. Ann Dermatol. 2011 Sep; 23(Suppl 1): S61–S63.
No treatment is needed. Reported successful interventions include topical retinoids, cryotherapy, and carbon dioxide laser ablation. Oral retinoids have also been variably beneficial, e.g. acitretin.
A 34-year-old woman presented with multiple asymptomatic discrete hyperpigmented and hypopigmented lesions over scalp, face, neck, trunk, and both limbs for 2 years. Indian J Dermatol 2016;61:237
Acrokeratosis verruciformis of Hopf in family. Indian Dermatol Online J 2014;5, Suppl S1:17-9
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