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Medical Definition of Poikiloderma atrophicans and cataract
1. The rothmund-thomson syndrome, a genetic disorder with numerous features affecting skin (premature aging, excess pigmentation, dilated blood vessels),eyes ( uvenile cataract), nose (saddle nose), teeth (maldeveloped), skeletal system (congenital bone defects) hair (abnormal), gonads (underdevelopment) limbs (soft tissue contractures), growth (short stature). Blood (anaemia) and a tendency to develop a type of bone cancer (osteogenic sarcoma). The rts gene is on chromosome 8. The syndrome is recessive so to be affected with rts a child has to have two rts genes, one from each parent. (12 Dec 1998)