Which clinical history findings are characteristic of café-au-lait pigmentation in McCune-Albright syndrome (MAS)?

Updated: Jan 17, 2019
  • Author: Gabriel I Uwaifo, MD; Chief Editor: George T Griffing, MD  more...
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Answer

Evaluation of café-au-lait pigmentation requires a detailed family history because neurofibromatosis (NF) also produces multiple café-au-lait spots. Unlike MAS, which occurs sporadically, NF is an autosomal dominant condition. A diagnosis of NF should be considered if a family history of café-au-lait pigmentation is noted, and the possibility should not be discounted even when precocious puberty occurs with café-au-lait spots. Hypothalamic optic gliomas with NF can lead to gonadotropin-dependent precocious puberty.


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