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Progressive multilayered banded skin in Winchester syndrome.

Authors :
Sidwell RU
Brueton LA
Grabczynska SA
Francis N
Staughton RC
Source :
Journal of the American Academy of Dermatology [J Am Acad Dermatol] 2004 Feb; Vol. 50 (2 Suppl), pp. S53-6.
Publication Year :
2004

Abstract

Winchester syndrome is a rare genetic disorder, one of the inherited osteolysis disorders which are a group of diseases characterized by destruction and resorption of affected bones with consequent skeletal deformities and functional impairment. The syndrome is characterized by dissolution of carpal and tarsal bones with generalized osteoporosis, progressive joint contractures, short stature, peripheral corneal opacities, and coarse facial features, though there is variability within the clinical features. Phenotypic heterogeneity of cutaneous features are also reported to date of diffusely thickened leathery skin, hypertrichosis, patches of hyperpigmented, hypertrichotic leathery skin in annular or linear distribution, widespread acne, subcutaneous nodules, and gingival hypertrophy. We describe widespread progressive multilayered symmetrical restrictive banding of the skin developing in a woman with Winchester syndrome during her mid-twenties.

Details

Language :
English
ISSN :
0190-9622
Volume :
50
Issue :
2 Suppl
Database :
MEDLINE
Journal :
Journal of the American Academy of Dermatology
Publication Type :
Academic Journal
Accession number :
14726867
Full Text :
https://doi.org/10.1016/s0190-9622(03)02466-6