CEPHALOMETRIC ANALYSIS OF RAPP-HODGKIN SYNDROME

Rapp-Hodgkin syndrome (RHS) is a rare form of ectodermal dysplasia wit h variable involvement of the hair, eyes, sweat glands, nails, and tee th. Oral findings may include hypodontia, hypoplastic enamel, cleft li p/palate, and a pronounced midfacial hypoplasia. The objective of this study was to determine if the pronounced midfacial hypoplasia is the result of a true tissue hypoplasia or displacement of midfacial tissue s. We have identified a family in which three members, a mother and tw o daughters, display clinical features characteristic of RHS. Clinical and intraoral examination of these affected persons showed manifestat ions characteristic of RHS, and several new findings were observed, in cluding subcutaneous abscesses, palmoplantar keratosis, and keratotic lesions located on the chest and trunk. To assess the midfacial hypopl asia, lateral cephalometric analysis was performed on lateral headplat es. Results of the cephalometric analysis suggest that the midfacial h ypoplasia results from both a deficiency and a displacement of the max illa. These findings are significant because successful dental treatme nt of RHS relies upon accurate assessment of current and projected oro facial development, particularly for the skeletal relations of the max illa and mandible.