The distribution of bones in
polyostotic fibrous dysplasia is often unilateral and monomelic.
(5) This syndrome is characterized by the triad of
polyostotic fibrous dysplasia, ipsilateral skin pigmentation, and precocious puberty.
A special type of
polyostotic fibrous dysplasia is McCune-Albright syndrome, which is associated with precocious sexual development and cafe au lait spots on the skin.
(4)
Polyostotic fibrous dysplasia, along with endocrine abnormalities and cutaneous hyperpigmentation, is a component of Albright-McCune-Sternberg syndrome, a rare condition that primarily affects females.
It has been suggested that such lesions that are not associated with involvement of the extracranial skeleton represent a third type of
polyostotic fibrous dysplasia. [3]
[1] There are two primary categories of the disease: monostotic fibrous dysplasia involves a single bone and represents 70% of all cases;
polyostotic fibrous dysplasia involves multiple bones and makes up the remainder, [2] Also, approximately 3% of patients with fibrous dysplasia will experience McCune-Albright syndrome, in which the bony involvement is accompanied by skin lesions and endocrinopathy.