Acrokeratosis verruciformis of Hopf

4 April 2012, by KANITAKIS J. & CRIBIER B.

Acrokeratosis verruciformis of Hopf (AVH) is a rare, most often hereditary (familial) disease. It is an autosomal dominant condition which means that it can affect both men and women and that the child of a patient with the disorder has a one in two chance of having it too; however, some people develop the disease even though their parents did not have it. Some patients have AVH only, whilst others may have an associated inherited condition, the most common of which is Darier’s disease. AVH causes small, well-defined lumps in the skin which have a rough, warty surface, are flesh-coloured or darker and, although sometimes difficult to see, are obvious to the touch. They resemble flat warts and tend to develop on the backs of the hands, the fingers and feet, or more rarely on the forearms, legs, elbows and knees. They are present at birth or first appear during childhood and adolescence. There have also been rare cases of people developing the lesions in later life (up to the fifties and sixties). Some people have similar lesions on their palms and nail abnormalities, but their face, scalp and torso are normal. These lumps last indefinitely and can become more apparent if the skin is exposed to the sun for prolonged periods. Under exceptional circumstances, a skin cancer can develop on these lesions, which is why patients with AVH are advised to check their skin and report any changes they observe (increase in size, sores, bleeding, etc.) to their doctor.

AVH is diagnosed on the basis of the appearance of the lumps, a family history of the disorder and, if necessary, the results of a skin biopsy. Treatment is not mandatory but may be requested by the patient for cosmetic reasons. At the present time, there is no definitive cure and the existing treatments are aimed at destroying the lesions themselves. They can be frozen off, cut out with a scalpel of removed with a laser. Some oral vitamin A derivatives (retinoids) have also been tried but the results are inconsistent.

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