Schneiderman Prelims Vol-I. qxd

PSYCHOCUTANEOUS DISORDERS
Factitial dermatitis
Neurotic excoriations
SYNDROMES
ADULT (acro-dermato-ungual-lacrimal-tooth) syndrome –
closely resemble EEC syndrome; hypodontia, ectrodactyly,
obstruction of lacrimal ducts, onychodysplasia, freckling
Am J Med Genet 45:642–648, 1993
Ataxia telangiectasia – hyperpigmented macules,
hypopigmented macules, café au lait macules
BJD 144:369–371, 2001
Bannayan–Riley–Ruvalcaba–Zonana syndrome –
hemangiomas, genital hyperpigmentation, supernumerary
nipples 
AD 132:1214–1218, 1996; Am J Med Genet
44:307–314, 1992
Cafe au lait macules (CALMs) and temporal dysrhythmia
Centrofacial lentiginosis 
Ghatan p.6, 2002, Second Edition
Cronkhite–Canada syndrome – lentigo-like macules of face,
extremities, and diffuse pigmentation of palms; gastrointestinal
polyposis, malabsorption, alopecia, dystrophic nails 
AD
135:212, 1999; Cutis 61:229–232, 1998
Familial multiple café au lait macules 
AD 130:1425–1426, 1994
Familial periorbital melanosis (hyperpigmentation) 
AD
100:169–174, 1969; Cutis 5:579, 1969
HYPERPIGMENTED LESIONS, DISCRETE ANNULAR
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Fanconi’s anemia – freckle-like hyperpigmentation in
sun-exposed areas, abdomen, flexures, and genitals
Dermatol Clin 13:41–49, 1995
Hermansky–Pudlak syndrome – freckling in sun-exposed skin
Rook p.1797, 1998, Sixth Edition; JAAD 19:217–255, 1988
Laugier–Hunziker syndrome
LEOPARD syndrome
Localized hereditary pruritus
Moynahan’s syndrome – lentigines, congenital mitral stenosis,
dwarfism, mental retardation, genital hypoplasia 
Ghatan p.6,
2002, Second Edition
Multiple mucosal neuroma syndrome (MEN IIB) – perioral or
periocular hyperpigmentation, lentigines, or freckles
NAME/LAMB syndromes; MEN IIA – hyperkeratosis and
hyperpigmentation in localized pruritic patch between the
scapulae 
JAAD 42:939–969, 2000
Neurofibromatosis type I – axillary freckling (Crowe’s sign)
J Med Genet 26:712–721, 1989; Ann Intern Med
61:1142–1143, 1964; CALMs
Peutz–Jeghers syndrome – lentigines; brown pigmented bands
of nails 
Ghatan p.78, 2002, Second Edition
Phakomatosis spilorosea (form of phakomatosis
pigmentovascularis) – nevus spilus with a telangiectatic nevus
AD 141:385–388, 2005
Piebaldism
Proteus syndrome – port wine stains, subcutaneous
hemangiomas and lymphangiomas, lymphangioma
circumscriptum, hemihypertrophy of the face, limbs, trunk;
macrodactyly, cerebriform hypertrophy of palmar and/or plantar
surfaces, macrocephaly; verrucous epidermal nevi, sebaceous
nevi with hyper- or hypopigmentation 
Am J Med Genet
27:99–117, 1987; vascular nevi, soft subcutaneous masses;
lipodystrophy, café au lait macules, linear and whorled macular
pigmentation 
Am J Med Genet 27:87–97, 1987; Pediatrics
76:984–989, 1985; Eur J Pediatr 140:5–12, 1983
Tuberous sclerosis – CALM
Watson’s syndrome – CALM and pulmonic stenosis
Westerhof syndrome – hypo and hypermelanotic macules on
the trunk and extremities
Xeroderma pigmentosum – acute sunburn, persistent erythema,
freckling – initially discrete, then fuse to irregular patches of
hyperpigmentation, dryness on sun-exposed areas; with time
telangiectasias and small angiomas, atrophic white macules
develop; vesiculobullous lesions, superficial ulcers lead to
scarring, ectropion; multiple malignancies; photophobia,
conjunctivitis, ectropion, symblepharon, neurologic
abnormalities 
Adv Genet 43:71–102, 2001; Hum Mutat
14:9–22, 1999; Mol Med Today 5:86–94, 1999; Derm Surg
23:447–455, 1997; Dermatol Clin 13:169–209, 1995; Recent
Results Cancer Res 128:275–297, 1993; AD 123:241–250,
1987; Ann Intern Med 80:221–248, 1974; XP variant AD
128:1233–1237, 1992