Ectodermal dysplasia in identical twins
A 23-year-old boy presented with a complaint of missing teeth with difficulty in speaking and eating to Farooqia Dental College and Hospital. He was moderately built, poorly nourished and had the typical face of EDA with features like: Frontal bossing, prominent supraorbital ridges, perioral pigmentation, dry skin, depressed nasal bridge, protuberant lips, low-set ears, recession of hair line all along with fine, sparse scanty scalp hair, scanty eyebrow and eye lashes. The left eye was slightly smaller than the right eye and the nails were not very brittle [Figures -]. Informed consent was procured from patients before any findings were reported. He was accompanied by his mother who gave a history of a total of six children born of a non-consanguineous marriage, with the boy and his younger male identical twin also suffering from the same condition since birth []. No other members of the family suffered from this trait. The mother also gave a history of the twins, presenting with partial anodontia in the upper jaw, complete anodontia of the lower jaw, progressive hyper pigmentation around eyes and mouth, complete absence of sweating since birth, repeated episodes of unexplained hyperpyrexia and thirst, which necessitated consistent drinking of cold water. They were of normal intelligence.\nMedical history revealed that the boy complained of frequent headache, dry eyes and poor vision in the left eye. Other systems were within normal limits. Intraoral examination revealed partial anodontia of the maxillary arch with the presence of four teeth suggestive of maxillary central incisors and canines on either side of the midline. The mandibular arch was completely edentulous with poorly developed alveolus. Moderate dryness of the mouth, with inflamed mucosa was evident []. A panoramic radiograph showed the presence of four cone-shaped crowns with roots in the maxilla consistent with central incisors and canines on either side of the midline. The mandibular alveolar ridge was narrow with complete anodontia and with evidence of right impacted canine like tooth [].\nThe younger twin brother also presented with almost the same identical physical and medical findings. He demonstrated good vision with no marked asymmetry of the eyes and a slight increase in scalp hair. Intraoral examination was identical. Examination of the panoramic radiographs of the younger twin showed the presence of four cone-shaped crowns with roots in maxilla consistent with central incisors and canines on either side of the midline. The mandibular teeth were missing with a narrow alveolar ridge. Cephalogram revealed normal growth pattern in both the twins []. Examination of impression and working model [] confirmed it. Since hypohidrosis, hypotrichosis and hypodontia were very evident on physical examination, the twins were diagnosed with hereditary hypohidrotic ectodermal dysplasia (HED) with partial anodontia. Treatment plan consisted of fixed partial dentures (FPD) for the conical shaped maxillary anterior teeth followed by construction of removable dentures.