Cartilage-hair hypoplasia (CHH) is one of the well-known immuno-osseous dysplasias (IOD), which are a combination of skeletal dysplasia and immunodeficiency. It is characterized by disproportionate short stature, fine sparse hair, ligamentous laxity, hematological abnormalities with anemia, a predisposition to malignant tumors, and recurrent infections usually due to cellular and/or humoral imm...

INTRODUCTION Mazabraud's syndrome is a rare but well-described disorder characterized by fibrous dysplasia in single or multiple bones associated with one or more soft-tissue myxomas. In this report, we describe what is, to the best of our knowledge, the first case involving an intra-osseous myxoma. This finding supports, and could provide new insight into, the pathological association between ...

OBJECTIVES Osseous dysplasias are the most common subtype of fibro-osseous lesions of the maxillofacial bones. They are benign and often present as incidental asymptomatic lesions. Diagnosis can be made with clinical and radiographic examination. CASE REPORT This article reports the case of a 47-year-old man with a huge cementifying osseous dysplasia of the maxilla that presented with seconda...

Florid cemento-osseous dysplasia (FCOD) has been described as a condition that characteristically affects the jaws of middle-aged black women. This condition has also been classified as gigantiform cementoma, chronic sclerosing osteomyelitis, sclerosing osteitis, multiple estenosis and sclerotic cemental masses. It usually exhibits as multiple radiopaque cementum-like masses distributed through...

Results All patients had characteristic poikiloderma as well as thumb anomalies. They were born dysmaturely and presented with failure to thrive. Age at genetic diagnosis was 5y, 4y and 3y for P1, P2, P3. Osteopenia and abnormal metaphyseal trabeculation of bones were striking on the initial skeletal survey in all patients. Z-scores on DXA scan were -0.1, -1.1 and -1.2 for P1, P2, P3 respective...

Cement-osseous dysplasia is a disease of localized fibrotic connective tissue proliferation accompanying cementum or bone-like hard at the tooth apex, while simple bone cyst specific featuring cavity without epithelial lining. Here, we report case cement-osseous associated with multiple cysts resembling cavities. A 60-year-old woman was referred to our hospital due complaint recurring pain and ...

BACKGROUND Fibro-osseous lesion is a broad term for a diverse group of jaw disorders characterized by the replacement of normal bone by a benign fibrous connective tissue matrix. The present study reviewed the clinico-pathologic characteristics of all biopsied cases of fibro-osseous lesions at our hospital from 1990 to 2011, which we hope will serve as a reference database regionally. METHODS...

Florid osseous dysplasia (FOD) is a rare fibro-osseous lesion of the jaw usually identified incidentally on radiograph. It rarely presents with clinical symptoms. A case of FOD presenting with features similar to osteomyelitis is discussed here. The diagnosis is based on radiographic findings; biopsy or surgical intervention should be avoided as it may predispose to infection.