TEETH
635
hypodontia, short upper lip bound down by multiple frenulae;
nail dystrophy, hair may be normal or sparse and brittle; cardiac
defects; ichthyosis, palmoplantar keratoderma
Ped Derm
18:485–489, 2001; J Med Genet 17:349–356, 1980; J Dent
Child 47:28–31, 1980; Arch Dis Child 15:65–84, 1940
FG syndrome (unusual facies, mental retardation, congenital
hypotonia, imperforate anus)
Am J Med Genet 19:383–386, 1984
Frontometaphyseal dysplasia – oligodontia
Radiol Clin North
Am 10:225–243, 1972
GAPO (growth retardation, alopecia, pseudoanodontia, optic
atrophy) – frontal bossing, midface hypoplasia
Birth Defects
24:205–207, 1988
Goltz’s syndrome
Hair–nail–skin–teeth dysplasias (dermo-odonto-dysplasia, pilo-
dento-ungular dysplasia, odonto-onycho-dermal dysplasia,
odonto-onychial dysplasia, tricho-dermo-sysplasia with dental
alterations)
Am J Med Genet 14:335–346, 1983
Hallermann–Streiff syndrome – partial anodontia, short stature,
atrophy and telangiectasia of central face, parrot-like
appearance, microphthalmia, cataracts, high-arched palate,
small mouth, sutural alopecia
JAAD 50:644, 2004
Hay–Wells syndrome (AEC syndrome)
BJD 94:277–289, 1976
Hemimaxillofacial dysplasia (segmental odontomaxillary
dysplasia) (HATS – hemimaxillary enlargement, asymmetry of
face, skin findings) – facial asymmetry, hypertrichosis of the
face, unilateral maxillary enlargement, partial anodontia,
delayed eruption of teeth, gingival thickening of affected
segment, Becker’s nevus, hairy nevus (hypertrichosis), lip
hypopigmentation, depression of cheek, erythema, hypoplastic
teeth
Ped Derm 21:448–451, 2004; JAAD 48:161–179, 2003;
Oral Surg Oral Med Oral Pathol 64:445–448, 1987
Hypodontia, sensorineural hearing loss and dizziness
Arch
Otolaryngol 104:292–293, 1978
Hypodontia, taurodontism, sparse hair
Birth Defects 11:39–50,
1975; Oral Surg 33:841–845, 1972
Hypoglossia–hypodactylia
Syndromes of the Head and Neck,
p.666–670, 1990
Hypohidrotic ectodermal dysplasia
Helv Paediatr Acta
11:604–639, 1956
Hypomelanosis of Ito – anodontia, dental dysplasia
Ghatan
p.267, 2002, Second Edition
Kindler’s syndrome – severe periodontitis with premature loss of
teeth
AD 140:939–944, 2004
Incontinentia pigmenti – pegged, conical teeth; partial
anodontia; delayed dentition
JAAD 47:169–187, 2002; J Pediatr
576:78–85, 1960
Jakac–Wolf syndrome – palmoplantar keratoderma with
squamous cell carcinoma, gingival dental anomalies,
hyperhidrosis
JAAD 53:S234–239, 2005
Johanson–Blizzard syndrome – hypodontia, microcephaly,
hypoplastic alae nasi, hearing loss, pancreatic dysfunction,
mental retardation
Birth Defects 24:205–207, 1988
KID syndrome – dental abnormalities
Ped Derm 19:232–236,
2002
LADD syndrome
Eur J Pediatr 146:536–537, 1987
Microcephaly, short stature, characteristic facies – oligodontia
Syndromes of the Head and Neck, p.871–872, 1990
Monosuperocentroincisivodontic dwarfism
J Pediatr
91:924–928, 1977
Mulvihill–Smith syndrome (premature aging, multiple nevi,
mental retardation) – oligodontia
J Med Genet 31:707–711,
1994; J Med Genet 25:53–56, 1988
Nevus sebaceous syndrome (Schimmelpenning–Feuerstein–
Mims syndrome) – anodontia, dysodontia
JAAD 52:S62–64,
2005; Ped Derm 13:22–24, 1996; Int J Oral Maxillofac Surg
12:437–443, 1983
Oligodontia, keratitis, skin ulceration and arthroosteolysis
Am J
Med Genet 15:205–210, 1983
Otodental dysplasia
Clin Genet 8:136–144, 1975
Pili torti, defective teeth, webbed fingers
JAAD 46:301–303, 2002
Pili torti, enamel hypoplasia syndrome – keratosis pilaris, dry
fair hair, enamel hypoplasia, widely spaced abnormal teeth
BJD
145:157–161, 2001
Pseudoacromegaly – autosomal recessive; skin ulcers,
arthro-osteolysis, keratitis, oligodontia
Am J Med Genet
15:205–210, 1983
Rapp–Hodgkin syndrome
J Med Genet 5:269–272, 1968
Reticulolinear aplasia cutis congenita of the face and neck –
Xp deletion syndrome, MIDAS (microphthalmia, dermal aplasia,
sclerocornea), MLS (microphthalmia and linear skin defects),
and Gazali–Temple syndrome; lethal in males; residual facial
scarring in females, short stature, organ malformations
BJD
138:1046–1052, 1998
Rieger syndrome – hypodontia and primary mesodermal
dysgenesis of the iris
Trans Am Ophthalmol Soc 81:736–784,
1983
Rutherfurd syndrome – autosomal dominant; gum hypertrophy,
failure of tooth eruption, corneal opacities, mental retardation,
aggressive behavior
Ped Derm 18:534–536, 2001
Schopf–Schulz–Passarge syndrome (hypotrichosis,
palmoplantar hyperkeratosis, apocrine hidrocystomas of eyelid
margins) – oligodontia
AD 140:231–236, 2004; JAAD
10:922–925, 1984
Trichodental syndrome – fine short hair, madurosis
BJD
116:259–263, 1987
Trichodento-osseous syndrome – curly hair, sclerotic cortical
bone, thin dental enamel, unerupted teeth
Oral Surg
77:487–493, 1994
Tuomaala–Haapanen syndrome (brachymetapody, anodontia,
hypotrichosis, albinoid trait)
Acta Ophthalmol 46:365–371, 1968
Waardenburg syndrome – caries
Ghatan p.267, 2002,
Second Edition
Witkop tooth–nail syndrome – hypodontia with nail dysgenesis
Oral Surg 37:576–582, 1974
X-linked anhidrotic ectodermal dysplasia
Birth Defects
24:205–207, 1988
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