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Progressive multilayered banded skin in Winchester syndrome.
- 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.
- Subjects :
- Abnormalities, Multiple diagnosis
Abnormalities, Multiple pathology
Adult
Collagen Diseases pathology
Contracture pathology
Diagnosis, Differential
Disease Progression
Dwarfism
Female
Hand Deformities, Acquired diagnosis
Hand Deformities, Acquired pathology
Humans
Pigmentation Disorders pathology
Syndrome
Thorax
Collagen Diseases diagnosis
Contracture diagnosis
Pigmentation Disorders diagnosis
Skin pathology
Subjects
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