Acrokeratosis Verruciformis of Hopf

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Acrokeratosis verruciformis of Hopf (or simply acrokeratosis verruciformis: AKV) is a rare genodermatosis characterized by keratotic lesions on the dorsum of the hands and feet. AKV most commonly develops during early childhood, but occasionally develops as late as the fifth decade. The natural history of the disease is a chronic course without spontaneous remission. It exhibits an autosomal dominant inheritance pattern and shows incomplete penetrance. Because of this, there may not always be a family history. There is no gender predilection.

Hopf first described AKV in 1931. The probable etiology is a mutation in the ATP2A2 gene located on chromosome 12q24,, which is the same gene affected in Darier disease. The diseases are distinct but may coexist in the same patient.

Clinically acrokeratosis verruciformis of Hopf appears as flat-topped keratotic papules and plaques on the dorsum of the hands and feet. Less frequently, the lesions may occur on the legs or arms. The disease spares sebaceous areas, like the frontal scalp, flexural surfaces, and oral mucosa. There are associated nail changes to include thickening of the nail plate, leukonychia, and longitudinal ridges.

Histopathologically, the lesions will have papillomatosis, acanthosis, hyperkeratosis, and hypergranulosis. The lesions will not have parakeratosis.