Distichiasis–lymphedema syndrome – double row of eyelashes,
nuchal webbing
BJD 142:148–152, 2000; AD 135:347–348,
1999; Clin Dysmorphol 3:139–142, 1994; Hum Genet
39:113–116, 1977
Dysplasia epiphysealis hemimelica (Trevor disease,
tarsomegaly) – edema of the feet
Episodic non-toxic erythema – swelling of the extremities
followed by generalized tender sunburn-like erythema, followed
by exfoliation
AD 132:1387–1388, 1996
Familial Mediterranean fever – edema with or without erythema
of the foot
AD 134:929–931, 1998
Fluid retention syndrome – idiopathic edema
Gardner–Diamond syndrome (painful bruising syndrome) –
ecchymoses of arms and legs preceded by edema and
erythema
JAAD 27:829–832, 1992; Blood 10:675–690, 1955;
autosensitization to DNA
Ann Intern Med 60:886–891, 1964
Hennekam’s syndrome – lymphedema, intestinal
lymphangiectasia, facial anomalies, mental retardation
Am J
Med Genet 34:593–600, 1989
Hereditary angioneurotic edema
Hurler’s syndrome and other mucopolysaccharidoses
Hypohidrotic ectodermal dysplasia of Zonana with osteopetrosis
and lymphedema – lymphedema; mutation in stop codon of
NEMO
Nat Genet 27:277–285, 2001
Idiopathic retroperitoneal fibrosis
Intestinal lymphangiectasia, pes cavus, microcephaly –
lymphedema
Klippel–Feil anomaly
Lymphedema-distichiasis syndrome
Ped Derm 19:139–141, 2002
Lymphedema of pubertal onset (Meige type) with cleft palate
Cleft Palate J 20:151–157, 1983
Lymphedema with recurrent cholestasis
West J Med
137:32–44, 1982
Lymphedema with intestinal angiectasia
West J Med
137:32–44, 1982
Melkersson–Rosenthal syndrome – orofacial edema
Microcephaly–lymphedema syndrome – with short stature
Am J Med Genet 280:506–509, 1998
Microcephaly-lymphedema-chorioretinal dysplasia syndrome –
edema of upper and lower extremities; autosomal dominant
or recessive
Ped Derm 22:373–374, 2005
Mulvihill–Smith syndrome – congenital lymphedema
Am J Med
Genet 69:56–64, 1997
Neu–Laxova syndrome – variable presentation; mild scaling to
harlequin ichthyosis appearance; ichthyosiform scaling,
increased subcutaneous fat and atrophic musculature,
generalized edema and mildly edematous feet and hands,
absent nails; microcephaly, intrauterine growth retardation, limb
contractures, low-set ears, sloping forehead, short neck; small
genitalia, eyelid and lip closures, syndactyly, cleft lip and palate,
micrognathia; autosomal recessive; uniformly fatal
Ped Derm
20:25–27,78–80, 2003; Curr Prob Derm 14:71–116, 2002;
Am J Med Genet 35:55–59, 1990
Neurofibromatosis – hemihypertrophy
Rook p.2287, 1998,
Sixth Edition
Neuronal migration defect and cerebellar hypoplasia –
lymphedema
Noonan’s syndrome – webbed neck, short stature, malformed
ears, nevi, keloids, transient lymphedema, ulerythema
ophyrogenes, keratosis follicularis spinulosa decalvans
JAAD
46:161–183, 2002; Rook p.3016, 1998, Sixth Edition; Ped Derm
15:18–22, 1998; J Med Genet 24:9–13, 1987; extremities Cutis
46:242–246, 1990
Papular–purpuric gloves and socks syndrome
Ped Derm
15:413, 1998
Peho syndrome – optic atrophy, progressive encephalopathy,
hypsarrhythmia
POEMS syndrome (Takatsuki syndrome, Crowe-Fukase
syndrome) – edema, osteosclerotic bone lesions, peripheral
polyneuropathy, hypothyroidism, and hypogonadism;
sclerodermoid changes (thickening of skin), either generalized
or localized (legs), cutaneous angiomas, blue dermal papules
associated with Castleman’s disease (benign reactive
angioendotheliomatosis), diffuse hyperpigmentation,
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
Pseudohypoparathyroidism – dry, scaly, hyperkeratotic puffy
skin; multiple subcutaneous osteomas, collagenoma
BJD
143:1122–1124, 2000
Reflex sympathetic dystrophy
JAAD 35:843–845, 1996; JAAD
28:29–32, 1993; AD 127:1541–1544, 1991
Scalenus anticus syndrome – indurated edema
Rook
p.2512–2513, 1998, Sixth Edition
Thrombocytopenia–absent radius syndrome (TAR syndrome) –
edema of feet
Trisomy 21 – congenital lymphedema
Hum Reprod 14:823–826,
1999
Trisomy 18 – congenital lymphedema
Hum Reprod 14:823–826,
1999
Trisomy 13 – congenital lymphedema
Hum Reprod 14:823–826,
1999
Turner’s syndrome – congenital lymphedema of upper and
lower extremities and neck, shield chest, wide nipples,
micrognathia, low hairline, webbed neck, pigmented nevi, short
stature
Ped Derm 22:27–275, 2005; JAAD 50:767–776, 2004;
lymphedema of scalp may mimic cutis verticis gyrata
Ped Derm
15:18–22, 1998
Wells’ syndrome (eosinophilic cellulitis)
AD 139:933–938, 2003;
BJD 140:127–130, 1999; AD 133:1579–1584, 1997; JAAD
33:857–64, 1995; JAAD 18:105–114, 1988; Trans S.Johns Hosp
Dermatol Soc 51:46–56, 1971
Yellow nail syndrome – lymphedema
Acta DV (Stockh)
63:554–555, 1983
TOXINS
Dieffenbachia picta (Dumb-cane) – chewing the leaves results
in perioral edema, salivation, burning, mucosal edema, and
blisters causing hoarseness or aphonia
Cutis 66:333–334, 2000
Eosinophilia myalgia syndrome (
L
-tryptophan related) –
erythematous and edematous rashes, peripheral edema,
morphea, urticaria, papular lesions; arthralgia
BJD 127:138–146,
1992; Int J Dermatol 31:223–228, 1992; Mayo Clin Proc
66:457–463, 1991; Ann Intern Med 112:758–762, 1990
TRAUMA
Childbirth – scalp edema (caput succedaneum) – due to
prolonged or precipitous delivery
AD 135:697–703, 1999
Dental treatment – soft tissue cervicofacial emphysema after
dental treatment
AD 141:1437–1440, 2005
Hematoma
Lymph node excision
Neonatal cold injury – facial erythema or cyanosis; firm pitting
edema of extremities spreads centrally resulting in total body
edema; skin is cold; mortality of 25%
Rook p.482, 1998,
Sixth Edition; Br Med J 1:303–309, 1960
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