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  • Title: Dental management of Rapp-Hodgkin syndrome associated with oral cleft and hypodontia.
    Author: Karthikeyani S, Thirumurthy VR, Yuvaraja BA.
    Journal: J Indian Soc Pedod Prev Dent; 2016; 34(2):192-5. PubMed ID: 27080974.
    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.
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