Other sclerosing dysplasias including, osteopathia striata, melorheostosis, and focal sclerosis can be seen. Lesions do not have increased bone radiotracer uptake. T1 weighted MRI reveals multiple well circumscribed low signal foci usually in a symmetric distribution. The differential diagnosis includes blastic mets, tuberous sclerosis and mastocytosis. However, symmetry and uniform size of lesions would be most suggestive of osteopoikilosis.
Histopathologic examination reveals numerous thickened collagen fibers in the dermis.
Orcein stain of the reticular layer of the dermis reveals numerous elastic fibers with various diameters. These frequently are fragmented, and in some places, they create a net of bundles. Weigert stain of the reticular dermis also reveals numerous elastic fibers with different diameters. In some patients, surgical treatment of deafness is possible. Surgical treatment of associated conditions such as stenosis of aortae should be performed as appropriate.