Overview
Fibrous dysplasia is a congenital, noninherited, benign intramedullary bone lesion in which the normal bone marrow is replaced by abnormal fibro-osseous tissue.
It can result in pain, deformity, fractures, or abnormalities in bone mechanics.
This condition was first described in 1942 by Lichtenstein and Jaffe
; hence, fibrous dysplasia is also sometimes referred to as Lichtenstein-Jaffe disease. The disorder can be monostotic (involving a single bone) or polyostotic (involving multiple bones).
When fibrous dysplasia occurs in the setting of other extraskeletal abnormalities, skin pigmentation, and endocrine dysfunction, the resulting syndrome is named McCune-Albright syndrome.
Fibrous dysplasia has been reported in association with several endocrinopathies, such as hyperthyroidism, hyperparathyroidism, acromegaly, diabetes mellitus, and Cushing syndrome.
Rarely, it can also be associated with solitary or multiple soft-tissue myxomas (Mazabraud syndrome).
The image below depicts distinctive pigmentation that can be seen in patients with fibrous dysplasia and endocrine abnormalities. Note that the border of the lesion is jagged and irregular.
Fibrous Dysplasia Pathology. This clinical photograph shows the distinctive pigmentation in patients with endocrine abnormalities associated with fibrous dysplasia.
See Fibrous Dysplasia Imaging for complete information about imaging on this topic.