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 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.
Sidwell
Rachel U
RU
Department of Dermatology (Hammersmith Hospitals Trust), Chelsea and Westminster Hospital, 369 Fulham Road, London SW10 9NH, UK.
Brueton
Louise A
LA
Grabczynska
Sophie A
SA
Francis
Nick
N
Staughton
Robert C D
RC
D000015:Abnormalities, Multiple / Q000175:diagnosis / Q000473:pathology; D000328:Adult; D003095:Collagen Diseases / Q000175:diagnosis* / Q000473:pathology; D003286:Contracture / Q000175:diagnosis* / Q000473:pathology; D003937:Diagnosis, Differential; D018450:Disease Progression; D004392:Dwarfism; D005260:Female; D006227:Hand Deformities, Acquired / Q000175:diagnosis / Q000473:pathology; D006801:Humans; D010859:Pigmentation Disorders / Q000175:diagnosis* / Q000473:pathology; D012867:Skin / Q000473:pathology*; D013577:Syndrome; D013909:Thorax