Juvenile hyaline fibromatosis [Murray-Puretic-Drescher Syndrome] oral, systemic and histopathological findings in an Egyptian girl

Juvenile hyaline fibromatosis [JHF] is an autosomal recessive condition that usually presents with nodular/ papular skin lesions and gingival hypertrophy during the first few years of life. There are several conditions in which gingival fibromatosis is seen as the oral finding and can be correlated with systemic or extraoral findings many of which have a genetic basis. This study describes clinical, genetic, orodental and histopathologic changes in a girl with features of the rare juvenile hyaline fibromatosis. Up to our knowledge this is the first recorded case in Egypt. We will discuss the major aspects of this syndrome and its differential diagnosis from other syndromes that may be associated with hereditary gingival fibromatosis