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SYNDROMES
Acquired sporadic generalized lentiginosis
Eur J Dermatol
8:183–185, 1998
ANOTHER syndrome – alopecia, nail dystrophy, ophthalmic
complications, thyroid dysfunction, hypohidrosis, ephelides and
enteropathy, respiratory tract infections
Clin Genet 35:237–242,
1989; J Pediatr 108:109–111, 1986
Bandler syndrome – autosomal dominant, Peutz–Jegher-like
lentigines; gastrointestinal bleeding with hemangiomas of small
intestine
Bolognia p.982, 2003
Bannayan–Riley–Ruvalcaba syndrome (macrocephaly
and subcutaneous hamartomas) (lipomas and hemangiomas) –
autosomal dominant pigmented macules of penis
JAAD
53:639–643, 2005; AD 132:1214–1218, 1996; AD
128:1378–1386, 1992; Eur J Ped 148:122–125, 1988;
lipoangiomas (perigenital pigmented macules, macrocephaly)
AD 128:1378–1386,
1992; lipomas in Ruvalcaba–Myhre–Smith syndrome Ped
Derm 5:28–32, 1988; Ruvalcaba–Myhre–Smith syndrome –
pigmented penile macules, lipomas, angiolipomas,
macrocephaly, pseudopapilledema, hamartomas, lipid-storage
myopathy
AD 132:1214–1218, 1996; Curr Prob Derm
VII:143–198, 1995; Pediatrics, 81:287–290, 1988
Cantu syndrome – autosomal dominant, lentigines on palms
and soles, and sun-exposed skin, palmoplantar hyperkeratotic
papules
Curr Prob Derm VII:143–198, 1995
Carney complex (NAME/LAMB syndrome) – autosomal
dominant, multiple lentigines, melanocytic nevi, blue nevi,
cutaneous myxomas, psammomatous schwannoma, cardiac
myxomas, testicular Sertoli cell tumors, gynecomastia, myxoid
breast fibroadenomas, pituitary adenomas, thyroid disease,
adreno-cortical disease
Molec Genet Metab 78:83, 2003;
Clin Endocrinol 86:4041, 2001; Curr Prob Derm VII:143–198,
1995; Medicine 64:270, 1985; conjunctival lentigines JAAD
42:145, 2000; epithelioid blue nevus and psammomatous
melanotic schwannoma
Semin Diagn Pathol 15:216–224, 1998;
J Clin Invest 97:699–705, 1996; Dermatol Clin 13:19–25, 1995;
JAAD 10:72–82, 1984
Centrofacial lentiginosis – synophrys, high arched palate,
sacral hypertrichosis, spina bifida, scoliosis
Rook p.1719, 1998,
Sixth Edition; BJD 94:39–43, 1976
Cowden’s disease – periorificial and acral pigmented macules
Bolognia p.982, 2003
Cronkhite–Canada syndrome – lentigo-like macules of face,
extremities, and diffuse pigmentation of palms; gastrointestinal
polyposis, malabsorption, alopecia, dystrophic nails
AD 135:212, 1999
Deafness
Bolognia p.982, 2003
FACES syndrome (unique facies, anorexia, cachexia, eye, skin
lesions)
J Craniofac Genet Dev Biol 4:227–231, 1984
Fanconi’s anemia – freckle-like hyperpigmentation in
sun-exposed areas, abdomen, flexures, and genitals
Dermatol Clin 13:41–49, 1995
Gardner’s syndrome – lentigines of head and extremities
JAAD 45:940–942, 2001
Gastro-cutaneous syndrome – peptic ulcer/hiatal hernia,
multiple lentigines, café-au-lait macules, hypertelorism, myopia
Am J Med Genet 11:161–176, 1982
Gaucher’s disease – diffuse hyperpigmentation, easy tanning,
pigmented macules
BJD 111:331–334, 1984
Halal syndrome – autosomal dominant; multiple lentigines,
café-au-lait macules, hypertelorism, myopia, hiatal hernia/peptic
ulcer
Am J Med Genet 11:161–176, 1982
Hermansky–Pudlak syndrome – multiple lentigines and
ephelides
Inherited patterned lentiginosis in blacks – lentigines on face
and lips, buttocks and extremities
AD 125:1231–1235, 1989
Laugier–Hunziker syndrome – macular pigmentation of lips and
buccal mucosa, melanonychia, genital macules
JAAD
50:S70–74, 2004; J Eur Acad Dermatol Venereol 15:574–577,
2001; Hautarzt 42:512–515, 1991; Clin Exp Derm 15:111–114,
1990; Arch Belges Dermatol Syph 26:391–399, 1970
Lentiginosis with arterial dissection syndrome
NEJM
332:576–579, 1995
Lentiginosis with cutaneous myxomas
JAAD 44’282–284, 2001
Lentiginosis with osteochondromyxoma of bone
Am J Surg
Pathol 25:164–1776, 2001
Lentiginosis with nevoid hypopigmentation
BJD 144:188–189,
2001
Lentiginosis with testicular microlithiasis
Clin Exp Dermatol
25:655–656, 2000
LEOPARD syndrome (multiple lentigines syndrome; Moynahan
syndrome) – autosomal dominant; generalized lentiginosis,
especially over neck and trunk; structural cardiac abnormalities,
cardiac symptoms, electrocardiographic abnormalities,
genitourinary abnormalities (gonadal hypoplasia, hypospadias,
delayed puberty), neurologic defects, cephalofacial
dysmorphism, short stature or low birth weight, skeletal
Schneiderman page 300-400.qxd 5/16/2006 6:50 PM Page 301
abnormalities
Ped Derm 21:139–145, 2004; JAAD 46:161–183,
2002; J Med Genet 34:582–586, 1997; Am J Dis Child
117:652–662, 1969
Neurofibromatosis – axillary freckling
Moynahan’s syndrome – lentigines, congenital mitral stenosis,
dwarfism, mental retardation, genital hypoplasia
Ghatan p.6,
2002, Second Edition
Mucocutaneous pigmentation with intestinal hemangiomatosis –
Peutz–Jegher-like lentiginosis with intestinal hemangiomatosis
Gastroenterology 38:641–645, 1960
Mukamel syndrome – autosomal recessive; premature graying in
infancy, lentigines, depigmented macules, mental retardation,
spastic paraparesis, microcephaly, scoliosis
Bolognia p.859, 2003
Multiple mucosal neuroma syndrome (MEN IIB) – perioral or
periocular lentigines, freckles, or hyperpigmentation
Noonan–like syndrome, cherubism and polyarticular villonodular
synovitis
Oral Surg Oral Med Oral Pathol 67:698–705, 1989
Peutz–Jeghers syndrome – autosomal dominant; oral lentigines
of gums, buccal mucosa, hard palate, lips, around nose and
mouth, palms and soles, dorsal hands and feet; lentigines fade
with time except mucosal lesions; gastrointestinal polyposis,
clubbing, ovarian tumors, precocious puberty with hormone
secreting tumors
Curr Prob Derm 14:41–70, 2002; Cancer
Res 58:5267–5270, 1998; Curr Prob Derm VII:143–198, 1995;
Gut 30:1588–1590, 1989; NEJM 316:1511–1514, 1987;
Gastroenterology 32:434–451, 1957; NEJM 241:992–1005,
1949
Phakomatosis pigmentokeratotica – speckled lentiginous nevus
in association with nevus sebaceus
Dermatology 197:377–380,
1998
Piebaldism – penile lentigines
Pipkin’s syndrome – autosomal dominant, nystagmus,
strabismus
Bolognia p.982, 2003
Progeria – axillary freckling
Ghatan p.239, 2002,
Second Edition
Progressive cardiomyopathic lentiginosis
Ped Derm 1:146–153,
1953
Tay’s syndrome – autosomal recessive
Bolognia p.982, 2003
Touraine centrofacial lentiginosis – autosomal dominant,
lentigines in butterfly distribution and forehead, central nervous
system abnormalities, high forehead, high-arched palate,
pectus deformities, kyphosis, spina bifida, umbilical hernias
BJD
94:39–43, 1976
Watson’s syndrome – café au lait macules, axillary and perianal
freckling, pulmonic stenosis, low intelligence, short stature
JAAD 46:161–183, 2002; JAAD 40:877–890, 1999
Xeroderma pigmentosum
BJD 152:545–551, 2005; AD
123:241–250, 1987
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