Sclerodactyly, hereditaryQ87.2

Author:Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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DefinitionThis section has been translated automatically.

Rare sclerosis of the fingers (toes), which leads to bending contracture already in childhood.

EtiopathogenesisThis section has been translated automatically.

Inheritance probably autosomal dominant with low penetrance.

ManifestationThis section has been translated automatically.

Occurring in early childhood. No further progression after the age of 20.

Clinical featuresThis section has been translated automatically.

Thickening of the fingers and possibly the toes and sclerotic skin. In the final stage, bending contracture of the fingers (or toes) II-V. Partially circumscribed hyperkeratoses of the palmae. No Raynaud's phenomenon, no fingertip defects.

Differential diagnosisThis section has been translated automatically.

Systemic scleroderma (acroscleroderma); camptodactyly; Dupuytren's contracture.

TherapyThis section has been translated automatically.

Possibly operative measures by hand surgeons.

LiteratureThis section has been translated automatically.

  1. Eubel R et al (1985) Hereditary sclerodactyly and syndactyly. Dermatologist 36: 302-324

Authors

Last updated on: 29.10.2020

Author: Prof. Dr. med. Peter Altmeyer