Keratosis palmoplantaris with scleratrophyQ82.8

Author:Prof. Dr. med. Peter Altmeyer

All authors of this article

Last updated on: 29.10.2020

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Synonym(s)

Scleroatrophic and keratotic dermatosis of limbs; Sclerotylosis

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HistoryThis section has been translated automatically.

Huriez, 1968

DefinitionThis section has been translated automatically.

Hereditary palmoplantar keratosis.

EtiopathogenesisThis section has been translated automatically.

Autosomal-dominantly inherited mutations of the sclerotylosis gene (HRZ gene; TYS gene; gene locus: 4q23 TYS).

ManifestationThis section has been translated automatically.

Disease may be present at birth.

Clinical featuresThis section has been translated automatically.

  • Discrete sometimes lamellar keratoses with atrophy, which diffusely cover the palms. Skin of the soles of the feet is less affected.
  • Associated are sclerodactyly (scleroderma-like), nail changes and hypohidrosis.

HistologyThis section has been translated automatically.

Histological changes are unspecific; epidermal Langerhans cells are missing in the affected skin.

TherapyThis section has been translated automatically.

Externally according to the keratosis palmoplantaris diffusa circumscripta. Response to systemic retinoids. Retinoids can apparently prevent or delay the development of carcinomas.

Progression/forecastThis section has been translated automatically.

Development of squamous cell carcinomas in the infected skin. High frequency of metastasis!

LiteratureThis section has been translated automatically.

  1. Guerriero C et al (2000) Huriez syndrome: case report with a detailed analysis of skin dendritic cells. Br J Dermatol 143: 1091-1096
  2. Huriez Cl, Agache P, Bombart M, Souilliart F (1963) Epitheliomas spinocellulaires sur atrophie cutanee congenitale dans deux familles a morbidite cancereuse elevee. Bull Soc Fr Dermatol Syph 70: 24-28
  3. Huriez C, Deminatti M, Agache P, Mennecier M (1968) Talking about the 28 cas d'epidermolysis bulleuse dans 11 familles dont une famille etudiee du point de une genetique, sans mise en evidence de linkage. Bull Soc Franc Derm Syph 75: 750-755
  4. Watanabe E et al (2003) A nonfamilial Japanese case of Huriez syndrome: p53 expression in squamous cell carcinoma. Dermatology 207: 82-84

Authors

Last updated on: 29.10.2020

Author: Prof. Dr. med. Peter Altmeyer