title: Dental management of Rapp-Hodgkin syndrome associated with oral cleft and hypodontia.
abstract: Rapp-Hodgkin syndrome (RHS) is a rare type of autosomal dominant disorder characterized by association of ectodermal dysplasia (ED) with cleft lip/palate. The main features include dry, brittle hair with alopecia in adulthood, dental anomalies (hypodontia, microdontia with delayed eruption, fissured tongue, and retruded maxilla), hypohidrosis, dysplastic nails, and clefting. Palmar-plantar keratoderma is seen frequently. RHS has signs and symptoms that overlap considerably with those of ankyloblepharon-ED-clefting syndrome and ectrodactyly-ED-clefting syndrome. This manuscript discusses a case of RHS, one of the four members in three generations who had ED with variable degree of involvement of hair, teeth, nail, and sweat glands.
authors.affiliation: Department of Pedodontics and Preventive Children Dentistry, Sri Ramakrishna Dental College and Hospital, Coimbatore, Tamil Nadu, India.
authors.forename: Shanmugasundaram
authors.initials: S
authors.lastname: Karthikeyani
authors.forename: Velliangattur Ramasamy
authors.initials: VR
authors.lastname: Thirumurthy
authors.forename: Bindhoo A
authors.initials: BA
authors.lastname: Yuvaraja
mesh_terms: D002508:Cephalometry; D002648:Child; D002971:Cleft Lip / Q000175:diagnosis* / Q000235:genetics* / Q000601:surgery; D002972:Cleft Palate / Q000175:diagnosis* / Q000235:genetics* / Q000601:surgery; D003241:Consanguinity; D003937:Diagnosis, Differential; D004476:Ectodermal Dysplasia / Q000175:diagnosis* / Q000235:genetics* / Q000601:surgery; D006801:Humans; D008297:Male; D008310:Malocclusion / Q000175:diagnosis* / Q000628:therapy; D010375:Pedigree; D011862:Radiography, Panoramic; D014071:Tooth Abnormalities / Q000175:diagnosis* / Q000628:therapy