and feet; atrophy with mottled hypo- and hyperpigmentation,
dermatomal CALMs, photosensitivity, canities, acanthosis
nigricans, dermatitis; cutaneous granulomas present as papules
or nodules, red plaques with atrophy or ulceration
Rook
p.2095,1998, Sixth Edition; JAAD 10:431–438, 1984; Ann Intern
Med 99:367–379, 1983
Berlin syndrome – no vellus hairs; mottled pigmentation and
leukoderma, flat saddle nose,
thick lips, fine wrinkling around
eyes and mouth (similar to Christ–Siemens ectodermal
dysplasia); stunted growth, bird-like legs, mental retardation
Dermatologica 123:227–243, 1961
Chediak–Higashi syndrome – speckled hyper- and
hypopigmentation; lysosomal trafficking regulator gene (Lyst);
defective microtubule-mediated lysosomal transfer
JAAD
49:S244–246, 2003
Congenital dyschromia with erythrocyte, platelet, and
tryptophan metabolic abnormalities
JAAD 19:642–655, 1988
Da Costa’s syndrome
Dermatopathia pigmentosa reticularis – generalized reticulate
hyperpigmentation; sweating disorders,
decreased
dermatoglyphics, alopecia, onychodystrophy, palmoplantar
keratoses
JAAD 50:S65–69, 2004
Dowling–Degos disease (reticulated pigmented anomaly of the
flexures) – dyschromatosis (reticulated pigmentation of axillae,
groin, and other intertrigenous areas), pitted atrophic scars at
corners and around mouth, freckles of vulva, comedo-like
lesions
BJD 147:568–571, 2002; JAAD 40:462–467, 1999;
Clin Exp Dermatol 9:439–350, 1984
Dyschromatosis symmetrica hereditaria (reticulate
acropigmentation of Dohi) – mutation in gene encoding
adenosine deaminase
AD 141:193–196, 2005; BJD
150:633–639, 2004; BJD 144:162–168, 2001; JAAD
43:113–117, 2000; Jpn J Dermatol 27:95–96, 1929; sun-
exposed areas only J
AAD 10:1–16, 1984
Dyschromatosis universalis with
small stature and high-tone
deafness
Clin Exp Derm 2:45–48, 1977
Dyschromatosis universalis hereditaria – autosomal dominant,
hyperpigmented macules in seborrheic regions, achromic macules
or diffuse hypopigmentation, leukotrichia
Ped Derm 19:523–526,
2002; Ped Derm 17:70–72, 2000; BJD 85:66–70, 1971
Dyskeratosis congenita (Zinsser–Cole–Engmann syndrome) –
X-linked recessive – cutaneous atrophy and pigmentation
J Med
Genet 12:339–354, 1975
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
Generalized mottled pigmentation
with postnatal blistering
JAAD 50:S65–69, 2004
Haber’s syndrome – persistent facial erythema, telangiectasias
with mild induration, similar to rosacea; darkly pigmented black
keratotic papules of axilla, neck, torso; pitted scars
HOPP syndrome – hypotrichosis, striate, reticulated pitted
palmoplantar keratoderma, acro-osteolysis, psoriasiform plaques,
lingua plicata, onychogryphosis, ventricular arrhythmias,
periodontitis
BJD 150:1032–1033, 2004; BJD 147:575–581, 2002
Hutchinson–Gilford syndrome (progeria) – mottled
hyperpigmentation
Am J Med Genet 82:242–248, 1999; J
Pediatr 80:697–724, 1972
Incontinentia
pigmenti
JAAD 50:S65–69, 2004
Mendes de Costa syndrome – generalized reticulate
hyperpigmentation on face and limbs, intraepidermal blisters,
microcephaly, mental retardation, atrichia, short conical fingers
JAAD 50:S65–69, 2004
Mitochondrial disorders – erythematous photodistributed eruptions
followed by mottled or reticulated hyperpigmentation; alopecia with
or without hair shaft abnormalities including trichothiodystrophy,
trichoschisis, tiger tail pattern, pili torti, longitudinal grooving, and
trichorhexis nodosa
Pediatrics 103:428–433, 1999
Naegeli–Franceschetti–Jadassohn syndrome – autosomal
dominant, punctate hyperpigmentation, waist, axillae, neck;
hypohidrosis, palmoplantar hyperkeratosis; tooth
enamel
hypoplasia and nail anomalies
JAAD 28:942–950, 1993; Clin
Exp Dermatol Jun;1(2):127–140, 1976
Photoleukomelanodermatitis of Kobori – dyschromic drug
eruption after treatment with afloqualone, thiazides, tetracycline
and ultraviolet exposure
Bolognia, p.1003, 2003
Reticulate acropigmentation of Kitamura – palmar pits
J Dermatol 27:745–747, 2000; JAAD 40:462–467, 1999
Unilateral dermatomal pigmentary dermatosis – segmental
dyschromatosis
JAAD 27:763–764, 1992
Wende–Bauckus/Pegum – zones of normal, yellow-bronze, and
black patches of hyperpigmentation; white
macules of trunk
Bolognia, p.1003, 2003
Werner’s syndrome (pangeria) – mottled hyperpigmentation
Medicine 45:177–221, 1966
Westerhof syndrome
Curr Prob Derm VII:143–198, 1995
X-linked reticulate pigmentary disorder with systemic
manifestations (familial cutaneous amyloidosis) (Partington
syndrome II) – X-linked; rare; Xp21–22; boys with generalized
reticulated muddy brown pigmentation (dyschromatosis) with
hypopigmented corneal dystrophy (dyskeratosis), coarse unruly
hair, unswept eyebrows, silvery hair, hypohidrosis, recurrent
pneumonia with chronic obstructive disease, clubbing; failure to
thrive, female carriers with linear macular nevoid Blascko-esque
hyperpigmentation
Ped Derm 22:122–126, 2005; Semin Cut
Med Surg 16:72–80, 1997; Am J Med Gen 10:65:1981
Xeroderma pigmentosum
Ped Derm 19:523–526, 2002;
resembling dyschromatosis symmetrica hereditaria
Ped Derm
3:410–413, 1986
Ziprkowski–Margolis syndrome –
X-linked recessive; pigmentary
dilution of hair and skin, sparing buttocks and genital areas; later
multiple hyperpigmented macules with leopard-like appearance;
deaf-mutism, heterochromic irides
Bolognia, p.1003, 2003
TOXINS
Arsenic – diffuse pigmentation, especially of trunk; with
depigmentation yielding rain-drop appearance
Rook p.1785,
1998, Sixth Edition
Mustard gas
AD 128:775–780, 1992
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