Journal of Indian Society of Pedodontics and Preventive Dentistry
Journal of Indian Society of Pedodontics and Preventive Dentistry
                                                   Official journal of the Indian Society of Pedodontics and Preventive Dentistry                           
Year : 2016  |  Volume : 34  |  Issue : 2  |  Page : 192--195

Dental management of Rapp-Hodgkin syndrome associated with oral cleft and hypodontia


Shanmugasundaram Karthikeyani1, Velliangattur Ramasamy Thirumurthy2, Bindhoo A Yuvaraja2 
1 Department of Pedodontics and Preventive Children Dentistry, Sri Ramakrishna Dental College and Hospital, Coimbatore, Tamil Nadu, India
2 Department of Prosthodontics, Sri Ramakrishna Dental College and Hospital, Coimbatore, Tamil Nadu, India

Correspondence Address:
Dr. Shanmugasundaram Karthikeyani
552, West Lokamanya Street, R.S. Puram, Coimbatore - 641 002, Tamil Nadu
India

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.


How to cite this article:
Karthikeyani S, Thirumurthy VR, Yuvaraja BA. Dental management of Rapp-Hodgkin syndrome associated with oral cleft and hypodontia.J Indian Soc Pedod Prev Dent 2016;34:192-195


How to cite this URL:
Karthikeyani S, Thirumurthy VR, Yuvaraja BA. Dental management of Rapp-Hodgkin syndrome associated with oral cleft and hypodontia. J Indian Soc Pedod Prev Dent [serial online] 2016 [cited 2021 May 13 ];34:192-195
Available from: https://www.jisppd.com/article.asp?issn=0970-4388;year=2016;volume=34;issue=2;spage=192;epage=195;aulast=Karthikeyani;type=0