abnormal ears and teeth, nail dystrophy, syndactyly, reduced
apocrine secretion
Bolognia p.924, 2003
Gardner’s syndrome – supernumerary teeth
Ghatan p.267,
2002, Second Edition
Hidrotic ectodermal dysplasia – ‘tiger teeth’
Ghatan p.267, 2002,
Second Edition
Hunter’s syndrome – widely spaced teeth; reticulated 2–10-mm
skin-colored papules over scapulae, chest, neck, arms; X-linked
recessive; MPS type II; iduronate-2 sulfatase deficiency;
lysosomal accumulation of heparin sulfate and dermatan
sulfate; short stature, full lips, coarse facies, macroglossia, clear
corneas (unlike Hurler’s syndrome), progressive
neurodegeneration, communicating hydrocephalus, valvular and
ischemic heart disease, lower respiratory tract infections,
adenotonsillar hypertrophy, otitis media, obstructive sleep
apnea, diarrhea, hepatosplenomegaly, skeletal deformities
(dysostosis multiplex), dolichocephaly, deafness, retinal
degeneration, inguinal and umbilical hernias
Ped Derm
21:679–681, 2004
Ichthyosis follicularis with atrichia and photophobia (IFAP) –
enamel dysplasia; collodion membrane and erythema at birth;
ichthyosis, spiny (keratotic) follicular papules (generalized
follicular keratoses), non-scarring alopecia, keratotic papules of
elbows, knees, fingers, extensor surfaces, xerosis; punctate
keratitis, photophobia; nail dystrophy, psychomotor delay, short
stature; beefy red tongue and gingiva, angular stomatitis, atopy,
lamellar scales, psoriasiform plaques, palmoplantar erythema
Curr Prob Derm 14:71–116, 2002; JAAD 46:S156–158, 2002;
BJD 142:157–162, 2000; Am J Med Genet 85:365–368, 1999;
Ped Derm 12:195, 1995; AD 125:103–106, 1989;
Dermatologica 177:341–347, 1988
Incontinentia pigmenti – anomalous crowns with extra cusps;
supernumerary teeth
JAAD 47:169–187, 2002
Jackli syndrome – generalized reticulated hyperpigmentation
with alopecia, microdontia, and childhood cataracts
Job’s syndrome (hyperimmunoglobulin E syndrome) – retention
of primary teeth with double rows of teeth; deep-set eyes, broad
nasal bridge, wide fleshy nasal tip, prognathism, ocular
hypertelorism
Clin Inf Dis 34:1213–1214,1267–1268, 2002;
JAAD S268–269, 2002; Pediatr 141:572–575, 2002; Curr Prob
Derm 10:41–92, 1998
Keratosis–ichthysosis–deafness (KID) syndrome – abnormal
teeth
Ped Derm 19:513–516, 2002
Minocycline – gray teeth
Ghatan p.267, 2002, Second Edition
Naegeli–Franceschetti–Jadassohn syndrome – autosomal
dominant; reticulate gray to brown pigmentation of neck, upper
trunk and flexures, punctate or diffuse palmoplantar
keratoderma, hypohidrosis with heat intolerance, onycholysis,
subungual hyperkeratosis, yellow tooth enamel
Ped Derm
22:122–126, 2005; JAAD 28:942–950, 1993
Oculodentodigital dysplasia – autosomal dominant; enamel
hypoplasia
Oral–facial–digital syndrome type I (Papillon–Leage syndrome) –
X-linked dominant; dental caries with widely spaced teeth; short
upper lip, hypoplastic alanasi, hooked pug nose, hypertrophied
labial frenulae, bifid or multilobed tongue with small tumors
within clefts, clefting of hard and soft palate, trident hand or
brachydactyly, syndactyly, or polydactyly; hair dry and brittle,
alopecic, numerous milia of face, ears, backs of hands, mental
retardation
Ped Derm 9:52–56, 1992
Osteogenesis imperfecta
Porphyria – congenital erythropoietic porphyria – erythrodontia
Rabson–Mendenhall syndrome – insulin-resistant diabetes
mellitus, unusual facies, dental precocity, fissured tongue,
hypertrichosis, acanthosis nigricans, and premature sexual
development
Ped Derm 19:267–270, 2002
Rapp–Hodgkin hypohidrotic ectodermal dysplasia – autosomal
dominant; alopecia of wide area of scalp in frontal to crown
area, short eyebrows and eyelashes, coarse wiry sparse
hypopigmented scalp hair, sparse body hair, scalp dermatitis,
ankyloblepharon, syndactyly, nipple anomalies, cleft lip and/or
palate; nails narrow and dystrophic, small stature, hypospadius,
conical teeth and anodontia or hypodontia; distinctive facies,
short stature
JAAD 53:729–735, 2005; Ped Derm 7:126–131,
1990; J Med Genet 15:269–272, 1968
Rothmund–Thomson syndrome – dental dysplasia
Ghatan
p.267, 2002, Second Edition
SHORT syndrome – short stature, hyperextensible joints, ocular
depression, Reiger (ocular and dental) anomaly, teething delay,
loss of subcutaneous fat of face, upper extremities, chest and
Sjögren–Larsson syndrome – autosomal recessive; enamel
dysplasia; serrated teeth
Ghatan p.177, 2002, Second Edition
Syphilis, congenital – Hutchinson’s incisors, mulberry molars
Tetracycline – discoloration of teeth
Tuberous sclerosis – enamel pits
JAAD 49:163–166, 2003;
Ghatan p.267, 2002, Second Edition
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