Acrokeratosis verruciformis Q82.8

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Hopf keratosis; Hopf syndrome

History
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Hopf, 1931

Definition
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Rare, autosomal-dominantly inherited cornification anomaly with Verrucae planae juveniles-like skin lesions. Questionable variant of dyskeratosis follicularis.

Etiopathogenesis
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Autosomal-dominantly inherited mutations of the ATP2A2 gene mapped on chromosome 12q23-24 are detectable (as in dyskeratosis follicularis). Therefore, some authors consider the clinical picture as a variant of dyskeratosis follicularis. Apparently mutations at different loci of the allele seem to cause the phenotypic expression of the clinical picture.

Manifestation
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Different start: Infancy to early adulthood.

Localization
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Preferably back of hands and feet, more rarely forearms, knees, elbows.

Clinical features
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Numerous lentil-sized, flat, skin-coloured to reddish-brown, mostly polygonal papules, some with warty surface. Confluence creates a cobblestone-like aspect. No pruritus.

Histology
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Differential diagnosis
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Therapy
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Keratolytically with vitamin A acid, tretinoin(e.g. R256, Cordes VAS) or salicylic acid 3-10% R227, if necessary under occlusion for a few hours . Alternatively: treatment withCO2 or Erbium-YAG laser or cryosurgery in open spray procedure.

Progression/forecast
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Transition into a spinocellular carcinoma possible.

Note(s)
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Joint appearance with M. Darier is possible.

Literature
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  1. Dhitavat J et al (2003) Acrokeratosis verruciformis of Hopf is caused by mutation in ATP2A2: evidence that it is allelic to Darier's disease. J Invest Dermatol 120: 229-232
  2. Dissemond J (2007) Generalized eczema and palmoplantar hyperkeratoses. dermatologist 58: 460-464
  3. Hafner O et al (1997) Acrokeratosis verruciformis-like changes in Darier disease. dermatologist 48: 572-576
  4. Hopf G (1931) On a previously undescribed disseminated keratosis (acrokeratosis verruciformis) Dermatol Zeitsch 60: 227-250
  5. Hopf G (1932) On the keratoses occurring on the hands and feet in Darier's disease. Acta Derm Venereol 13: 720-734

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Last updated on: 29.10.2020