Which of the following is not associated with cafe-au-lait spots?

Correct Answer: Von Hippel-Lindau Syndrome
Description: Cafe-au-lait spots are discrete macular skin lesions with light brown pigmentation that usually present within the first year of life. Common "bihmarks", they often occur as an isolated finding. The presence of five or more cafe-au-lait spots should prompt consideration of neurofibromatosis, one of the 4 or 5 neurocutaneous syndromes. Other disorders may be associated with cafe-au-lait spots. This includes multiple lentigines, ataxia telangiectasia, Turner's syndrome, tuberous sclerosis, Fanconi anemia, and Russell-Silver syndrome. Ref: Salant D.J., Gordon C.E. (2012). Chapter 284. Polycystic Kidney Disease and Other Inherited Tubular Disorders. In D.L. Longo, A.S. Fauci, D.L. Kasper, S.L. Hauser, J.L. Jameson, J. Loscalzo (Eds), Harrison's Principles of Internal Medicine, 18e.
Category: Medicine
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