Sclerodactyly

Classification according to ICD-10
L94.3	Sclerodactyly
Q87.2	Congenital malformation syndromes with predominant involvement of the extremities - hereditary sclerodactyly
ICD-10 online (WHO version 2019)

The Sclerodactyly , from ancient Greek σκληρός skleros , German , hard ' and ancient Greek δάκτυλος daktylos , German , finger' , a hardening (is sclerosis ) of the skin of the fingers. It is characterized by the taut and hardened skin over thin, pale and hairless fingers. Sclerodactyly occurs in systemic sclerosis and CREST syndrome as well as congenitally as hereditary sclerodactyly .

Synonym : acrosclerosis

distribution

The frequency is unknown; the hereditary form is likely to be inherited as an autosomal dominant trait.

Sclerodactyly is found in:

Scleroderma (about 25%)
CREST syndrome (about 5%), (calcinosis - Raynaud's phenomenon - oesophageal dysfunction - sclerodactyly - telangiectasia)

as well as for other diseases and syndromes :

Diabetes mellitus
Huriez syndrome (palmoplantar hyperkeratosis-sclerodactyly syndrome)
POEMS syndrome
Palmoplantar keratosis - congenital alopecia, autosomal recessive (cataract - alopecia - sclerodactyly)
Sharp syndrome (mixed collagenosis)
Celiac disease

and still questionable new syndromes.

Differential diagnosis

The following are to be distinguished:

literature

R. Eubel, L. Klose, G. Mahrle: Hereditary sclerodactyly and. Syndactyly. In: The dermatologist; Journal of Dermatology, Venereology, and Allied Fields. Volume 36, Number 5, May 1985, pp. 302-304, PMID 2989220 .

Individual evidence

^ Pschyrembel
↑ Encyclopedia Dermatology
↑ Hereditary Form Encyclopedia Dermatology
↑ ^a ^b Radiopaedia
^ LM Gruson, A. Franks: Scleredema and diabetic sclerodactyly. In: Dermatology online journal. Volume 11, Number 4, December 2005, p. 3, PMID 16403375 .
↑ Palmoplantar keratosis - congenital alopecia, autosomal recessive. In: Orphanet (Rare Disease Database).
^ I. Zammit-Maempel, AR Adamson, JP Halsey: Sclerodactyly complicating celiac disease. In: British journal of rheumatology. Volume 25, Number 4, November 1986, pp. 396-398, doi: 10.1093 / rheumatology / 25.4.396 , PMID 3779328 .
^ EG Lemire, P. Petch: Short stature, pulmonary hypertension, sclerodactyly-like involvement and dysmorphic appearance: a newly described syndrome? In: Clinical dysmorphology. Volume 13, Number 4, October 2004, pp. 227-230, doi: 10.1097 / 00019605-200410000-00005 , PMID 15365458 .
↑ C. Wallis, FS Ip, P. Beighton: Cataracts, alopecia, and sclerodactyly: a previously apparently undescribed ectodermal dysplasia syndrome on the island of Rodrigues. In: American journal of medical genetics. Volume 32, Number 4, April 1989, pp. 500-503, doi: 10.1002 / ajmg.1320320415 , PMID 2773993 .

Web links

Entry on sclerodactyly in the Flexikon , a Wiki of the DocCheck company

[pschy-1] Pschyrembel

[Altmeyer-2] Encyclopedia Dermatology

[3] Hereditary Form Encyclopedia Dermatology

[radio-4] Radiopaedia

[5] LM Gruson, A. Franks: Scleredema and diabetic sclerodactyly. In: Dermatology online journal. Volume 11, Number 4, December 2005, p. 3, PMID 16403375 .

[6] Palmoplantar keratosis - congenital alopecia, autosomal recessive. In: Orphanet (Rare Disease Database).

[7] I. Zammit-Maempel, AR Adamson, JP Halsey: Sclerodactyly complicating celiac disease. In: British journal of rheumatology. Volume 25, Number 4, November 1986, pp. 396-398, doi: 10.1093 / rheumatology / 25.4.396 , PMID 3779328 .

[8] EG Lemire, P. Petch: Short stature, pulmonary hypertension, sclerodactyly-like involvement and dysmorphic appearance: a newly described syndrome? In: Clinical dysmorphology. Volume 13, Number 4, October 2004, pp. 227-230, doi: 10.1097 / 00019605-200410000-00005 , PMID 15365458 .

[9] C. Wallis, FS Ip, P. Beighton: Cataracts, alopecia, and sclerodactyly: a previously apparently undescribed ectodermal dysplasia syndrome on the island of Rodrigues. In: American journal of medical genetics. Volume 32, Number 4, April 1989, pp. 500-503, doi: 10.1002 / ajmg.1320320415 , PMID 2773993 .