Sclerodactyly, hereditary Q87.2
Definition
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Rare sclerosis of the fingers (toes), which leads to bending contracture already in childhood.
Etiopathogenesis
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Inheritance probably autosomal dominant with low penetrance.
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Manifestation
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Occurring in early childhood. No further progression after the age of 20.
Clinic
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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 diagnosis
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Therapy
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Possibly operative measures by hand surgeons.
Literature
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- Eubel R et al (1985) Hereditary sclerodactyly and syndactyly. Dermatologist 36: 302-324
Outgoing links (6)
Camptodactyly; Dupuytren's contracture; Hyperkeratoses; Raynaud's syndrome; Scleroderma systemic; Sclerosis;;Disclaimer
Please ask your physician for a reliable diagnosis. This website is only meant as a reference.