PMC5941722_01
Patient 1. Child with hidrotic ED had sparse hair and eyebrows and discrete perioral pigmentation (a and b).
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Upon extraoral examination, perioral pigmentation and dry skin were evidenced (Figure 1).
Patient 1. Accentuated diastema between the upper central incisors due to insertion of the labial frenulum (a), mixed dentition with no caries (b), and absence of the 31, 41, 12, 22, 32, 42, 13, 23, 33, 43, 34, 44, 35, and 45 teeth (c).
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Fourteen permanent teeth were absent, including the right left lower central incisors, right left upper and lower lateral incisors, right left upper and lower canines, and right left lower second first premolars (Figure 2).
Patient 1. Histological sections showing hyperkeratosis (a), atresia of sweat glands and absence of sebaceous glands (b), and atrophic hair follicles (c) (original magnification: x200 and x100; hematoxylin and eosin).
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Thus, an incisional biopsy in the gluteal region was performed to confirm the diagnosis of ectodermal dysplasia, and the histological sections showed few hair follicles and eccrine sweat glands and absence of sebaceous glands (Figure 3).
PMC5941722_02
Patient 2. Boy with hypohidrotic ED displaying scarce, fine, and silky hair, alopecia of the eyebrows, prominent frontal bossing, discrete deformity and low implantation of the ear (a and b), angular cheilitis (arrow), dry and scaly skin, and perioral dermatitis and fissures (c).
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fig4
Upon extraoral examination, scarce, fine and silky hair, alopecia of the eyelashes and eyebrows, prominent frontal bossing, discrete bilateral deformity and low implantation of the ear, perioral dermatitis and fissures, bilateral angular cheilitis, periorbital pigmentation, and deep nasolabial sulcus were evidenced (Figure 4).
Patient 2. Agenesis of almost all permanent teeth, except the 13th tooth, underdeveloped alveolar ridge with "knife-edge" shape (a and b) and prosthodontic rehabilitation with the use of upper and lower dentures (c).
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fig5
Initially, the recommended treatment was to make provisional upper and lower dentures (Figure 5), restoring the aesthetics and masticatory functions and, as a consequence, improving the patient's facial appearance and psychological well being.
PMC5941722_03
Patient 3. Child with hypohidrotic ED presented sparse hair, perioral and nasal pigmentation, ear deformity with pointed shape (a and b), and hyperkeratosis on the palms and knee (c and d).
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fig6
Upon extraoral examination, a depressed nasal bridge ("saddle nose"), thin and scanty hair, alopecia of the eyelashes and eyebrows, dry and crusted eyes, periorbital, perioral, and nasal pigmentation, bilateral deformity and low implantation of the ear, perioral fissures, and deep nasolabial sulcus were evidenced (Figure 6).
Patient 3. (a) Upper and (b) lower alveolar ridges were edentulous and underdeveloped, and (c) radiographic images showing the presence of the 53rd, 63rd, 73rd, and 83rd teeth.
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The upper and lower alveolar ridges were underdeveloped (Figure 7).