PRIMARY CUTANEOUS DISEASES
Acanthosis nigricans, generalized
Ped Derm 18:213–216, 2001
Confluent and reticulated papillomatosis
Disseminated dermal melanocytosis
BJD 101:197–205, 1979
Epidermolysis bullosa simplex with mottled pigmentation;
may be generalized or of neck, upper trunk, arms and leg
with or without keratoderma (punctate keratoses); cutaneous
atrophy, nail dystrophy
Clin Genet 15:228–238, 1979
Human chimerae with pigment anomalies
Idiopathic eruptive macular hyperpigmentation
Ped Derm
13:274–277, 1996; JAAD 11:159, 1984
Melanism – autosomal dominant; diffuse hyperpigmentation,
especially of face and flexures
Bull Acadr Med Belg
13:397–428, 1948
SYNDROMES
Acquired generalized lentiginosis
Eur J Dermatol 8:183–185, 1998
Berlin syndrome – mottled pigmentation and leukoderma
Breast hypertrophy, erythema annulare centrifugum,
generalized melanoderma, verrucae vulgaris and SLE
Acta DV
(
Stockh) 52:33, 1972
Costello syndrome – diffuse hyperpigmentation; 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 with single palmar crease, gingival
hyperplasia, hypoplastic nails, moderately short stature,
craniofacial abnormalities, hyperextensible fingers, sparse curly
hair, perianal and vulvar papules, generalized hypertrichosis,
multiple nevi
Ped Derm 20:447–450, 2003; JAAD 32:904–907,
1995; Aust Paediat J 13:114–118, 1977
Cronkhite–Canada syndrome – diffuse hyperpigmentation with
accentuationn of face, neck, extremities, palms and palmar
aspects of fingers
Ann DV 112:951–958, 1985
Degos–Touraine syndrome – incontinentia pigmenti with
poikiloderma in photodistribution, bullae of face, extremities;
chronic erythroderma with subsequent hyperpigmentation
Soc
Gr Dermatol Syph 68:6–10, 1961
Dermatopathia pigmentosa reticularis
J Dermatol 24:266–269,
1997
Diffuse pigmentation of trunk and neck with subsequent white
macules
Proc R Soc Med 48:179–180, 1955
Diffuse pigmentation with macular depigmentation of trunk with
reticulate pigmentation of neck
Hautarzt 6:458–460, 1955
Dyschromatosis symmetrica hereditaria
Dyschromatosis universalis hereditaria
Ann DV 128:136–138,
2001
Dyskeratosis congenita – X-linked recessive; reticulate
hyperpigmentation (poikiloderma) of neck, chest, thighs; nail
dystrophy, oral, ocular, and anal leukoplakia
J Med Genet
25:843–846, 1988
Elejalde syndrome (neuroectodermal lysosomal disease) –
bronze skin, silver hair
AD 135:182–186, 1999
Epidermal melanocytosis – congenital generalized
hyperpigmentation
AD 86:412–418, 1962
Epidermolysis bullosa herpetiformis with mottled pigmentation
and palmoplantar keratoderma
AD 122:900–908, 1986
Familial pigmentation with nail dystrophy – congenital
generalized hyperpigmentation
AD 71:591–598, 1955
Familial progressive hyperpigmentation (congenital generalized
hyperpigmentation, hereditary universal melanosis)
AD
125:1442–1443, 1989; AD 103:581–588, 1971
Fanconi’s syndrome (pancytopenia with congenital defects) –
generalized olive-brown hyperpigmentation, especially of lower
trunk, flexures, and neck with depigmented macules (rain drop-
like); hypoplastic anemia, slender build, short broad thumbs,
tapered fingers, microcephaly, hypogonadism
Semin Hematol
4:233–240, 1967
Felty’s syndrome
Franceschetti–Jadassohn–Naegeli syndrome – generalized
reticulated hyperpigmentation, accentuated in neck and axillae;
palmoplantar keratoderma; hypohidrosis
JAAD 10:1–16, 1984
Hurler’s (MPS I), Hurler–Scheie, Scheie syndromes –
generalized hyperpigmentation and thickening of skin
Ped Derm
21:154–159, 2004
Incontinentia pigmenti
Koraxitrachitic syndrome – self-healing collodion baby with
residual dappled atrophy
Am J Med Genet 86:454–458, 1999
Lawrence–Seip syndrome (lipoatrophic diabetes)
J Dermatol
19:246–249, 1992; Acta DV 66:173–174, 1986
Neurocutaneous melanosis
Neurofibromatosis type I
Noonan’s syndrome – diffuse hyperpigmentation following
bilateral adrenalectomy
Rook p.1763, 1998, Sixth Edition
Pachyonychia congenita with cutaneous amyloidosis and
rippled hyperpigmentation
JAAD 16:935–940, 1987
Pachyonychia congenita, Tidman–Wells–MacDonald type
Parana hard skin syndrome (stiff skin syndrome)
Ped Derm
20:339–341, 2003; Ped Derm 19:67–72, 2002
Phakomatosis pigmentovascularis
POEMS syndrome (Takatsuki syndrome, Crowe–Fukase
syndrome) – generalized hyperpigmentation, osteosclerotic
bone lesions, peripheral polyneuropathy, hypothyroidism, and
hypogonadism, cutaneous angiomas, blue dermal papules
associated with Castleman’s disease (benign reactive
angioendotheliomatosis), diffuse hyperpigmentation,
morphea-like changes, maculopapular brown-violaceous
lesions, purple nodules
JAAD 44:324–329, 2001;
JAAD 40:808–812, 1999; Cutis 61:329–334, 1998 ;
JAAD 21:1061–1068, 1989; JAAD 12:961–964, 1985;
AD 124:695–698, 1988
Polyglandular autoimmune syndrome type II – Schmidt’s
syndrome
Proteus syndrome
Symmetric acropigmentation of Dohi (Addison’s disease, thyroid
disease with or without diabetes)
Cutis 59:77–80, 1997
Tuberous sclerosis – diffuse bronzing
Universal dyschromatosis, small stature, and high tone
deafness
Werner’s syndrome (pangeria) – diffuse hyperpigmentation
Medicine 45:177–221, 1966
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