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Abstract Tuberous Sclerosis. Presented by Dr. W. B. Reed. A patient, age 55, is of normal mentality. The history is negative for epilepsy. Dermatological manifestations consist of (1) adenoma sebaceum since age 8 years; (2) shagreen patch of the lumbosacral area; (3) multiple ungual fibromas of hands and feet since "teen" age. Removal was followed by regrowth.Roentgenogram of the skull was negative for calcification. On removal of a right ectopic kidney multiple hamartomas (angiomyolipomas) were found. The right eye has a phakoma. A bone survey showed "pagetoid changes;" fibrous displacement in pelvic bones, and cortical hyperostosis of phalanges.The family history revealed that a sister, age 63, had ungual fibromas, but this was denied by the patient.Past history of "rickets" and congenital dislocation of hips treated in childhood.A patient, 4 years, has a history of slow mental development and 2 severe attacks of status epilepticus
Archives of Dermatology – American Medical Association
Published: Jan 1, 1962
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