Progressive multilayered banded skin in Winchester syndrome

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...

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Published inJournal of the American Academy of Dermatology Vol. 50; no. 2; pp. 53 - 56
Main Authors Sidwell, Rachel U, Brueton, Louise A, Grabczynska, Sophie A, Francis, Nick, Staughton, Robert C.D
Format Journal Article
LanguageEnglish
Published New York, NY Mosby, Inc 01.02.2004
Elsevier
Subjects
Abnormalities, Multiple - diagnosis
Abnormalities, Multiple - pathology
Adult
Biological and medical sciences
Collagen Diseases - diagnosis
Collagen Diseases - pathology
Contracture - diagnosis
Contracture - pathology
Dermatology
Diagnosis, Differential
Disease Progression
Dwarfism
Female
Hand Deformities, Acquired - diagnosis
Hand Deformities, Acquired - pathology
Humans
Medical sciences
Pigmentation Disorders - diagnosis
Pigmentation Disorders - pathology
Skin - pathology
Syndrome
Thorax
Case study
Human
Skin disease
Association
Dermatology
Diseases of the osteoarticular system
Winchester syndrome
Genetic disease
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Summary: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.
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ISSN:0190-9622
1097-6787
DOI:10.1016/S0190-9622(03)02466-6