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A Study of Cases of Hereditory Ectodermal Dysplasia - A Rare Dental Anomaly
Authors:Arun Kumar.S.Bilodi  S. Bilodi  MR Gangadhar
Affiliation:1. Department of Anatomy Mahatma Gandhi Medical College & Research Institute, Puducherry;2. Department of Anthropology, Manasa Gangothri University of Mysore, Mysore;1. Telecommunications Laboratory (LABTEL), Electrical Engineering Department, Federal University of Espírito Santo, Fernando Ferrari avenue, 29075-910 Vitória, ES, Brazil;2. Instituto de Telecomunicações, Campus Universitário de Santiago, 3810-193 Aveiro, Portugal;1. Division of Urology, Obstetrics and Gynecology, United States Food and Drug Administration, Silver Spring, MD;2. Duke-Margolis Center for Health Policy, Washington, DC;3. University of Maryland Francis King Carey School of Law and Johns Hopkins Berman Institute of Bioethics, Baltimore, MD;4. Novartis Pharmaceuticals Corporation, East Hanover, NJ;5. Department of Obstetrics, Gynecology & Reproductive Sciences, University of California San Diego School of Medicine, San Diego, CA;6. Department of Social Medicine and Center for Bioethics, University of North Carolina at Chapel Hill, Chapel Hill, NC;7. Division of Pharmacology Toxicology for Rare Diseases, Pediatrics, Urology and Reproductive Medicine, United States Food and Drug Administration, Silver Spring, MD;8. Office of Pediatric Therapeutics, United States Food and Drug Administration, Silver Spring, MD;9. Division of Pediatric and Maternal Health, United States Food and Drug Administration, Silver Spring, MD;10. Office of Rare Diseases, Pediatrics, Urology and Reproductive Medicine, United States Food and Drug Administration, Silver Spring, MD;1. Department of Civil and Environmental Engineering, University of Pittsburgh, Pittsburgh, PA, USA;2. Center for Geospatial Analysis, William & Mary, Williamsburg, VA, USA;3. Alcoa Technical Center, New Kensington, PA, USA
Abstract:
Hereditary ectodermal dysplasia is a group of disorder running in the family where more than one manifestation occurs involving skin, nail, hair, glands and teeth In the present study, five cases were detected in district of Bangalore,Karnataka and studied in detail. Out of them three were girls and two boys showing manifestation dysplasia of teeth, skin & sweat glands between the age groups of 5 years to 14 years of age. There are three girls between 5 to 18 years showing oligodentia (0.13%) in 2 girls and anodentia in one girl (0.67%) associated with periorbital wrinkling and mild mid facial hypoplasia.The other 2 were boys between 8years and 15 years of age showed oligodentia, anhydosis brittle nails with vertical ridges, and 15 years old boy also showed periorbital wrinkling. The mothers of these five patients were also studied. Consanguity along with heredity and hypertension has played a vital role in the development of ectodermal dysplasia These 5 cases were compared and correlated with available literatures.
Keywords:Ectodermal dysplasia  Oligodontia  Anhydrosis  Consanguineous couples Sparse distribution of hair  xerostomia
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