In which of the following condition is an association of sexual precocity, multiple cystic bone lesions and endocrinopathies seen?
Correct Answer: McCune-Albright's syndrome
Description: McCune-Albright's syndrome is the condition which is normally associated with a triad of findings such as precocious pubey, cafe au lait spots and polyostotic fibrous dysplasia. It is also associated with hyperfunctioning endocrinopathies such as hypehyroidism, hypercoisolism, hypersomatotropism, and hypophosphatemic rickets. Affected children have cafe au lait spots which are flat, hyperpigmented skin lesions that have rough borders(coast of Maine), in contrast to the cafe-au-lait lesions of neurofibromatosis that have smooth borders(coast of California). Children affected by this syndrome presents at a younger age than children with idiopathic precocious pubey. Vaginal bleeding occurs early in these children and is the first sign of pubey. Ref: Lapeere H., Boone B., De Schepper S., Verhaeghe E., Van Geel M., Ongenae K., Van Geel N., Lambe J., Brochez L. (2012). Chapter 75. Hypomelanoses and Hypermelanoses. In K. Wolff (Ed), Fitzpatrick's Dermatology in General Medicine, 8e.
Category:
Pediatrics
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