Melorheostosis is a rare benign disease of cortical bone most frequently presenting as peripheral hyperostosis with a characteristic "melting wax" appearance on conventional radiographs. The disease most frequently affects the appendicular skeleton and is seen only rarely in the craniofacial bones. We discuss a case of melorheostosis in the nasal cavity and skull base with an atypical radiographic appearance and suggest findings that may differentiate craniofacial melorheostosis from more common entities in this region.
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Distinct Clinical and Pathological Features of Melorheostosis Associated With Somatic MAP2K1 Mutations.
J Bone Miner Res
January 2019
Clinical and Investigative Orthopedics Surgery Unit, National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Bethesda, MD, USA.
Melorheostosis is a rare hyperostotic disease of the long bones classically characterized by a "dripping candle-wax" radiographic appearance. We recently described somatic activating mutations in MAP2K1 as a cause of melorheostosis. Here, we report distinguishing characteristics of patients with MAP2K1-positive melorheostosis.
View Article and Find Full Text PDFMelorheostosis and central giant cell granuloma of the mandible in a 15-year-old girl.
Oral Surg Oral Med Oral Pathol Oral Radiol
November 2013
Department of Diagnostic Sciences and Oral Medicine, University of Tennessee College of Dentistry, Memphis, TN, USA. Electronic address:
Melorheostosis is a nonhereditary bone dysplasia primarily affecting the appendicular skeleton. Because clinical and histologic features are often nonspecific, the diagnosis is often based on the radiographic presentation. Involvement of the craniofacial skeleton is rare.
View Article and Find Full Text PDFCraniofacial melorheostosis.
J Comput Assist Tomogr
October 2008
Department of Radiology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA.
Melorheostosis is a rare benign disease of cortical bone most frequently presenting as peripheral hyperostosis with a characteristic "melting wax" appearance on conventional radiographs. The disease most frequently affects the appendicular skeleton and is seen only rarely in the craniofacial bones. We discuss a case of melorheostosis in the nasal cavity and skull base with an atypical radiographic appearance and suggest findings that may differentiate craniofacial melorheostosis from more common entities in this region.
View Article and Find Full Text PDFMelorheostosis: report of two cases affecting the jaw.
Oral Surg Oral Med Oral Pathol Oral Radiol Endod
October 2007
School of Dentistry, University of Colorado, Aurora, CO 80045, USA.
Melorheostosis is a rare sclerosing bone dysplasia that is characterized by a localized, diffuse thickening of the cortical bone. This condition usually affects the appendicular skeleton and associated soft tissue and rarely affects the craniofacial complex. The etiology of this condition is obscure.
View Article and Find Full Text PDFOsteosclerotic bone dysplasia (melorheostosis) of the mandible.
Int J Oral Maxillofac Surg
December 2006
Clinic for Oral and Maxillofacial Surgery, Kantonsspital Luzern, Luzern, Switzerland.
Melorheostosis is a linear bone dysplasia of unknown origin that may be associated with soft-tissue alterations. Although any part of the skeleton can be affected, this condition is rarely observed in the craniofacial region. Only seven cases of melorheostosis with craniofacial involvement have been reported and cranial manifestation only is even rarer.
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