Acrokeratosis Verruciformis of Hopf Clinical Presentation
- Author: Sarah Sweeney Pinney, MD; Chief Editor: William D James, MD more...
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, 16]
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.
Nail involvement, including longitudinal splitting, striations, and subungual hyperkeratosis, also may be seen.
Variants can also present on the trunk, as is seen in a case following the lines of Blaschko.
Acrokeratosis verruciformis of Hopf has an autosomal dominant mode of transmission. This has been suggested since 1962, in a large follow-up series by Niedleman and McKusick that described 24 cases in 4 generations in the same family.
Sporadic cases can also occur.
Acrokeratosis verruciformis and Darier disease are allelic disorders. ATP2A2 encoding the SERCA2 pump has been identified as the defective gene in Darier disease. In 2003, Dhitavat et al identified a heterozygous P602L mutation in the ATP2A2 gene in a family affected with acrokeratosis verruciformis for 6 generations. This mutation predicts a nonconservative amino acid substitution in the ATP-binding domain of the molecule. The mutation segregates with the disease phenotype in the family and was not found in 50 controls. Moreover, functional analysis of the P602L mutant showed that it has lost its ability to transport Ca2+. This result demonstrates loss of function of the SERCA2 mutant in acrokeratosis verruciformis, thus providing evidence that acrokeratosis verruciformis and Darier disease are allelic disorders.
The possible occurrence of squamous cell carcinoma in the context of the lesions of acrokeratosis verruciformis of Hopf has been rarely described, and one case report describes an association with keratoacanthomas.[22, 23]
This condition may also be associated with hypertrophic lichen planus and multiple steatocystomas.
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