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A cohort study found that 18 of 283 (6.4%) individuals with RDEB had a diagnosis of SCC with a median age of diagnosis of 22.6 years (interquartile range, 2027.7 y).[145] Early mortality also has been observed in this disorder, with a mortality rate of up to 40% by age 30 years.[146] An epidemiological study in Germany found that the mean age of death was 28.03 years in patients with DEB.[130] Extracutaneous manifestations of RDEB-sev gen include short stature, anemia, strictures of the gastrointestinal and genitourinary tracts, and corneal scarring that may result in blindness
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