Jaffe-Lichtenstein syndrome - Report of a rare case

Abstract

Fibrous dysplasia (FD) is a benign slow growing skeletal disorder that causes swelling of the bone. Lichtenstein described FD in 1938 as skeletal bone disorder which is distinguished by continuous replacement of normal bone with fibrous matrix. FD is also associated with Jaffe-Lichtenstein syndrome (JLS) and McCune-Albright syndrome (MAS). JLS is characterized by polyostotic FD along with café-au-lait pigmented lesions on the skin. MAS presents with additional features of endocrine disorders along with the FD. The disease is found to cause significant aesthetic problem and functional disturbance, particularly those affecting the craniofacial skeleton. Most often, the clinical features and the classic radiographic presentation of the pathology would be sufficient to make a diagnosis. However other fibro-osseous lesions that mimic the radiographic features are critical to make a differential diagnosis for the clinician. The present case report is about a 6 year old boy from south India who has been diagnosed as Jaffe-Lichtenstein syndrome (JLS). Keywords: Fibrous dysplasia, McCune-Albright Syndrome, Cafe-au-laithyperpigmentation, Lichtenstein-Jaffe’s disease, Osteitisfibrosa, alkaline phosphatase, Acid phospatase, Bone biopsy.