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Therapeutics in Dermatology
A reference textbook in dermatology
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Acrokeratosis verruciformis of Hopf

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

Acrokeratosis verruciformis of Hopf (AVH) was first described in 1931 [1] as an autosomal dominant abnormal keratinisation genodermatosis (OMIM 101900). It displays specific clinical and histological features, although almost indistinguishable lesions are found in 73% of patients with Darier disease (or their siblings) [2, 3], casting doubts on the nosological autonomy of AVH. According to some authors, the two conditions are distinct. Microscopically, the acral lesions in Darier disease show alterations characteristic of this disorder (acantholytic dyskeratosis), which does not exist in AVH [4] ; furthermore, the characteristic mutations of Darier disease, that affect the ATP2A2 gene (chromosome 12q23-q24), have been detected in some families with AVH [5], but not in Chinese ones with AVH [6]. The second hypothesis is that AVH is an allelic form of Darier disease since, in at least some cases [5], the same mutations of the ATP2A2 gene (whose expressivity is variable) are found in AVH. Sporadic cases of AVH and association with other conditions – such as Hailey-Hailey disease, Rothmund-Thomson syndrome, epidermolysis bullosa, sebocystomatosis, hypertrophic lichen planus, keratoacanthomas, nevoid basal cell carcinoma syndrome, psoriasis and cardiomyopathy [7-12]) have also been reported. While these associations may be fortuitous, it is possible that AVH is heterogeneous, and represents a clinical manifestation common to several diseases including – mainly – Darier disease [13].

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