Hypohidrotic ectodermal dysplasia: dental, clinical, genetic and dermatoglyphic findings of three cases.
Patients with hypohidrotic ectodermal dysplasia (HED) are characterized by the clinical manifestations of hypodontia, hypohidrosis, hypotrichosis and a highly characteristic facial physiognomy. This disorder is inherited as an X-linked trait. This report presents three cases with HED in which the clinical evaluation (intraoral and radiological), genetic findings and SEM examination of hair. Boys 6 to 14 year old and a 11 year old girl were referred to the Marmara University, Faculty of Dentistry, complaining of oligodontia in the maxillary and mandibular arches and delay in eruption of other teeth. Peg-shaped teeth have been observed. The dermatoglyphs of the patients were striking. SEM examination of hair demonstrated a distinctly abnormal longitudinal grooving along the entire length of each hair and a desquamation of the surface cuticles. The treatment was planned in a multidisciplinary odontological group involving pediatric dentistry, orthodontics, prosthodontics and oral surgery and maxillofacial radiology of future dental habilitation. A specially designed overdenture, a removable prosthesis and osseointegrated implants were constructed. Periodic recall visits were advised, to monitor the dentures and implants during periods of growth and development, and eruption of the permanent teeth.
Kargül
B
B
Department of Pediatric Dentistry, Dental School, Marmara University, Istanbul, Turkey. b.kargul.ped@marun.edu.tr
Alcan
T
T
Kabalay
U
U
Atasu
M
M
D000848:Anodontia / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D003758:Dental Implantation, Endosseous; D015921:Dental Implants; D019094:Dental Prosthesis, Implant-Supported; D003828:Denture, Overlay; D003832:Denture, Partial, Removable; D003878:Dermatoglyphics / Q000145:classification; D004476:Ectodermal Dysplasia / Q000235:genetics / Q000473:pathology* / Q000503:physiopathology; D019066:Facies; D005260:Female; D005500:Follow-Up Studies; D008040:Genetic Linkage; D006197:Hair / Q000002:abnormalities; D006801:Humans; D007007:Hypohidrosis / Q000473:pathology; D007039:Hypotrichosis / Q000473:pathology; D008297:Male; D008855:Microscopy, Electron, Scanning; D010347:Patient Care Planning; D010375:Pedigree; D014071:Tooth Abnormalities / Q000473:pathology; D014078:Tooth Eruption / Q000502:physiology; D014960:X Chromosome / Q000235:genetics
D015921:Dental Implants