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

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


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
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0970-4388.180453

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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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  2005 - Journal of Indian Society of Pedodontics and Preventive Dentistry | Published by Wolters Kluwer - Medknow 
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