SYNDROMES
Aarskog syndrome – single palmar crease
J Pediatr
77:856–861, 1970
Acral angiokeratoma-like pseudolymphoma (APACHE
syndrome) – linear red papules
JAAD S209–211, 2001
Acrocephalopolysyndactyly – linear submental scars
Alagille syndrome – linear palmar xanthomas
Amniotic band syndrome – linear erosions, crusts, and
constrictions
AD 130:1055–1060, 1994; Cutis 44:64–66, 1989
Anhidrotic ectodermal dysplasia – linear atrophic lesions of face
Ataxia telangiectasia – linear telangiectasias
Rook p.2095,1998,
Sixth Edition; Ann Intern Med 99:367–379, 1983
Auriculotemporal syndrome (Frey syndrome) – linear flush
and/or sweating on cheek after eating
Ped Derm 17:415–416,
2000; AD 133:1143–1145, 1997
Bannayan–Riley–Ruvalcaba–Zonana syndrome (PTEN
phosphatase and tensin homolog hamartoma) – transverse
palmar crease, dolicocephaly, frontal bossing, macrocephaly,
ocular hypertelorism, long philtrum, thin upper lip, broad mouth,
relative micrognathia, lipomas, penile or vulvar lentigines, facial
verruca-like or acanthosis nigricans-like papules, multiple
acrochordons, angiokeratomas, accessory nipple, syndactyly,
brachydactyly, vascular malformations, arteriovenous
malformations, lymphangiokeratoma, goiter, hamartomatous
intestinal polyposis
JAAD 53:639–643, 2005
Beckwith–Wiedemann syndrome (Exomphalos–Macroglossia–
Gigantism) (EMG) syndrome – autosomal dominant; linear
earlobe grooves, zosteriform rash at birth, exomphalos,
macroglossia, visceromegaly, facial salmon patch of forehead,
upper eyelids, nose, and upper lip and gigantism; circular
depressions of helices; increased risk of Wilms’ tumor, adrenal
carcinoma, hepatoblastoma, and rhabdomyosarcoma
JAAD
37:523–549, 1997; Am J Dis Child 122:515–519, 1971
Branchio-oculo-facial syndrome (dermal thymus resembling
linear scar) – autosomal dominant
Ped Derm 12:24–27,
1995; AD 125:1681–1684, 1989; Am J Med Genet 27:943–951,
1987
Brooke–Spiegler syndrome – linear papular eruption of eccrine
spiradenomas
Australas J Dermatol 44:144–148, 2003
Cantu syndrome – linear deep plantar creases, congenital
hypertrichosis, cardiomegaly, osteochondrodysplasia, coarse
facial features
Am J Med 92:191–194, 2000
Carbohydrate-deficient glycoprotein syndrome – emaciated
appearance; lipoatrophy over buttocks; lipoatrophic streaks
extend down legs; high nasal bridge, prominent jaw, large ears,
inverted nipples, fat over suprapubic area and labia majora, fat
pads over buttocks; hypotonia
Textbook of Neonatal
Dermatology, p.432, 2001
Carpenter syndrome – single palmar crease
Am J Med Genet
28:311–324, 1987
Carvajal syndrome – striate palmoplantar keratoderma with
woolly hair and cardiomyopathy
Bolognia p.757, 2003
Cerebro-hepato-renal syndrome (Zellweger syndrome) – single
palmar crease
Am J Med Genet 22:419–426, 1985
CHILD syndrome – congenital hemidysplasia, ichthyosis,
limb defects, ichthyosiform erythroderma with verruciform
xanthoma, linear red waxy scaling eruptions, and
hypopigmented bands
Ped Derm 15:360–366, 1998;
xanthomatous pattern
Dermatologica 180:263–266, 1990; AD
123:503–509, 1987
Chromosome deletion Xp22.1 – Gazali–Temple syndrome
JAAD 31:680–682, 1994
vs. Adams–Oliver syndrome
Aicardi syndrome
Cutis marmorata telangiectatica congenita
Goltz syndrome
Cohen syndrome – single palmar crease
J Med Genet
17:430–432, 1980
Congenital hypertrophy of the retinal pigment epithelium – linear
hyperpigmentaion
Am J Med Genet 126A:89–92, 2004
Congenital ichthyosiform dermatosis with linear keratotic
flexural papules and sclerosing palmoplantar keratoderma
AD 125:103–106, 1989
Conradi–Hünermann syndrome – linear hyperkeratotic bands
with diffuse erythema and scale, follicular atrophoderma,
hypochromic areas, scalp alopecia
Ped Derm 15:299–303,
1998; AD 127:539–542, 1991; Hum Genet 53:65–73, 1979
Cornelia de Lange syndrome – single palmar flexion crease;
specific facies, hypertrichosis of forehead, face, back,
shoulders, and extremities, synophrys; long delicate eyelashes,
cutis marmorata, skin around eyes and nose with bluish tinge,
red nose
Ped Derm 19:42–45, 2002; Rook p.428, 1998,
Sixth Edition; JAAD 37:295–297, 1997; Am J Med Genet
20:453–459, 1985
Costello syndrome – linear deep palmar creases; warty papules
around nose and mouth, legs, perianal skin; loose skin of neck,
hands, and feet; acanthosis nigricans; low set protuberant ears,
thick palmoplantar surfaces, gingival hyperplasia, hypoplastic
nails, moderately short stature, craniofacial abnormalities,
hyperextensible fingers, sparse curly hair, perianal and vulvar
papules, diffuse hyperpigmentation, generalized hypertrichosis,
multiple nevi
Ped Derm 20:447–450, 2003; JAAD 32:904–907,
1995; Am J Med Genet 47:176–183, 1993; Aust Paediat
J 13:114–118, 1977
Craniodystosis with extremity bands
Delleman–Oorthys syndrome
Downs’ syndrome (trisomy 21) – Simean crease
Syndromes
of the Head and Neck, p.35, 1990
EEC syndrome – linear cleft; p63 mutation
BJD 146:216–220,
2002
Ehlers–Danlos syndrome types I, II, and III – linear scars
Rook p.2034, 1998, Sixth Edition
Encephalocranial lipomatosis – linear yellow papules
of forehead extending to eyelids; ophthalmologic
manifestations; seizures, mental retardation; mandibular
or maxillary ossifying fibromas and odontomas
Ped Derm
22:206–209, 2005; hairless, pink to yellow tumors of scalp in
linear configuration; atrophic hairless patches of scalp and face
Ped Derm 10:164–168, 1993
Epidermodysplasia verruciformis
BJD 121:463–469, 1989;
Arch Dermatol Res 278:153–160, 1985
Fabry’s disease – punctate and linear angiectasis
AD 131:81–86, 1995; linear perioral telangiectasias
AD 126:1655–1656, 1990
Faciogenitopopliteal (popliteal–pterygium) syndrome – linear
fibrous cord of leg with ulcer
AD 124:1443–1444, 1988
Familial transverse nasal hyperpigmentation
J Hered
65:157–159, 1974
Glucagonoma syndrome – linear band along waistband
Rook
p.2728, 1998, Sixth Edition; AD 133:909, 912, 1997;
JAAD 12:1032–1039, 1985; Ann Intern Med 91:213–215,
1979
Goldenhaar syndrome – multiple linear accessory tragi
JAAD 50:S11–13, 2004
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