Monostotic Fibrous Dysplasia and Ichthyosis Vulgaris ------ A Rare Association

Fibrous Dysplasia of bone is an uncommon congenital skeletal disorder that is found equally in both genders and is not inherited. Fibrous Dysplasia is characterized either monostotic or polyostotic, and may occur as a component of McCune Albright Syndrome or the rare Mazabraud Syndrome. Long bones, skull bones and ribs are most commonly affected bones. 1 We present a case of a 18 years old boy having pathological fracture of right shaft femur due to Fibrous Dysplasia associated with Ichthyosis Vulgaris. Clinicoradiological examination further established our diagnosis. Biopsy stated delicate trabeculae of immature bone, with no osteoblastic rimming, enmeshed within a bland fibrous stroma of dysplastic spindle shaped cells without any cellular features of malignancy.