Amelo-cerebro-hypohidrotic syndrome (Kohlschutter
syndrome) – X-linked or autosomal recessive; hypohidrosis,
hypoplastic yellow tooth enamel, epilepsy, spasticity, mental
retardation
Helv Paediatr Acta 29:283–294, 1974
Anhidrotic ectodermal dysplasia (Christ–Siemens–Touraine
syndrome)
J Dermatol 26:44–47, 1999; X-linked recessive –
absent or reduced sweating, hypotrichosis, and total or partial
anodontia with conical pointed teeth
J Med Genet 28:181–185,
1991; autosomal recessive Ped Derm 7:242, 1990
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
Aplasia of sweat glands
Rook p.1997, 1998, Sixth Edition
Atopic dermatitis
Bazex–Dupre–Christol syndrome
J Med Genet 33:493–497,
1996; Clin Exp Dermatol 6:31–41, 1981; Bazex–Dupre–
Christol-like syndrome – basal cell carcinomas, hypohidrosis,
hypotrichosis, milia
Derm Surg 26:152–154, 2000
Cockayne syndrome – anhidrosis
Congenital absence of eccrine glands
Congenital erosive and vesicular dermatosis with reticulate
scarring
JAAD 45:946–948, 2001; Ped Derm 15:214–218, 1998;
JAAD 32:873–877, 1995
Congenital ichthyosis, follicular atrophoderma, hypotrichosis,
and hypohidrosis
Am J Med Geneet 13:186–189, 1998
Congenital insensitivity to pain with anhidrosis – secondary
acquired generalized anhidrosis
BJD 150:589–593, 2004
Congenital poikiloderma, traumatic bullae, anhidrosis,
keratoderma
Acta DV 59:347–351, 1979
Dermal inflammatory conditions
Dermatopathia pigmentosa reticularis – autosomal dominant,
reticulate hyperpigmentation of trunk, onychodystrophy,
alopecia, oral hyperpigmentation, punctate hyperkeratosis of
palms and soles, hypohidrosis; atrophic macules over joints
with hypertrophic scarring
Semin Cut Med Surg 16:72–80,
1997; AD 126:935–939, 1990; Hautarzt 6:262, 1960
Diabetes mellitus secondary acquired generalized anhidrosis
BJD 150:589–593, 2004
Ectodermal dysplasias, including EEC syndrome (ectrodactyly,
ectodermal dysplasia, and cleft palate)
BJD 94:277–289, 1976
Ectodermal dysplasia with cataracts and hearing defects
(Marshall’s syndrome) – dental defects, cataracts, deafness
Am J Ophthalmol 45:143–156, 1958
Ectodermal dysplasia – ankyloblepharon, absent lower
eyelashes, hypoplasia of upper lids, coloboma, seborrheic
dermatitis, cribriform scrotal atrophy, ectropion, lacrimal duct
hypoplasia, malaligned great toenails, gastroesophageal reflux,
ear infections, laryngeal cleft, dental anomalies, scalp hair
coarse and curly, sparse eyebrows, xerosis, hypohidrosis, short
nose absent philtrum, flat upper lip
BJD 152:365–367, 2005
Exfoliative dermatitis
Clin Exp Derm Mar 3 (1):99–101, 1978
Fabry’s disease – secondary acquired generalized anhidrosis
AD 140:1440–1446, 2004; BJD 150:589–593, 2004
Familial simple hypohidrosis with abnormal palmar dermal
ridges
Am J Med Genet 31:591–596, 1988
Franceschetti–Jadassohn–Naegeli syndrome – generalized
reticulated hyperpigmentation, accentuated in neck and axillae;
palmoplantar keratoderma; hypohidrosis
JAAD 10:1–16, 1984
Hutchinson–Gilford syndrome (progeria)
Am J Med Genet
82:242–248, 1999; J Pediatr 80:697–724, 1972
Hydroxyurea – long term therapy
Rinsho Kesueki
41:1214–1219, 2000
Hypohidrosis and diabetes insipidus (Fleck syndrome) –
hypohidrosis, hypotrichosis, diabetes insipidus, syndactyly,
coloboma, disturbed hematopoiesis
Dermatol Wochenschr
132:994–1007, 1955
Hypohidrosis with neurolabyrinthitis (Helwig–Larsen–Ludwigsen
syndrome)
AD 95:456–459, 1967
Hypohidrotic ectodermal dysplasia
Syndromes of the Head and
Neck, p.451, 1990
Hypoplastic enamel–onycholysis–hypohidrosis
(Witkop–Brearley–Gentry syndrome) – marked facial
hypohidrosis, dry skin with keratosis pilaris, scaling and crusting
of the scalp, onycholysis and subungual hyperkeratosis,
hypoplastic enamel of teeth
Oral Surg 39:71–86, 1975
Ichthyosis – congenital ichthyosiform erythroderma
Rook
p.1997, 1998, Sixth Edition
Idiopathic acquired generalized anhidrosis
BJD 150:589–593,
2004; Dermatologica 178:123–125, 1989; J Neurol
235:428–431, 1988
Incontinentia pigmenti – third stage; anhidrotic and achromic
areas
JAAD 47:169–187, 2002; BJD 116:839–849, 1987
Inflammatory or hyperkeratotic conditions
Kirman syndrome – anhidrosis, almost total alopecia, severe
mental retardation
BJD 67:303–307, 1953
Leprosy – lepromatous, borderline – anhidrosis overlying
plaques
Rook p.1225, 1998, Sixth Edition
Lichenoid drug eruption – sequela
Rook p.1917, 1998,
Sixth Edition
Lymphoma – adnexotropic (syringotropic) T-cell lymphoma –
generalized anhidrosis, alopecia, pruritus, Sjögren’s
syndrome
BJD 151:216–226, 2004; JAAD 38:493–497,
1998; BJD 130:765–769, 1994; BJD 110:95–101, 1984;
Proc R Soc Med 62:157–159, 1969
Miliaria
Morphea
Rook p.2504–2508, 1998, Sixth Edition
Myelomatosis
Rook p.1997, 1998, Sixth Edition
Naegeli syndrome–Franceschetti–Jadassohn syndrome
JAAD 28:942–950, 1993; Clin Exp Dermatol Jun;1 (2):127–140,
1976; Naegeli–Franceschetti–Jadassohn syndrome variant –
reticulate pigmentary dermatosis with hypohidrosis and short
stature
Int J Dermatol 34:30–31, 1995
Necrobiosis lipoidica diabeticorum
BJD 108:705–709, 1983
Neonatal anhidrosis
Rook p.1997, 1998, Sixth Edition
Olmsted syndrome – periorificial keratotic plaques; congenital
diffuse sharply marginated transgradient keratoderma of palms
and soles, onychodystrophy, constriction of digits, diffuse
alopecia, thin nails, chronic paronychia, leukokeratosis of oral
mucosa, linear keratotic streaks, follicular keratosis, constriction
of digits (ainhum), anhidrosis, small stature; differential
diagnostic considerations include Clouston hidrotic ectodermal
dysplasia, pachyonychia congenita, acrodermatitis
enteropathica, Vohwinkel’s keratoderma, mal de Meleda, and
other palmoplantar keratodermas
Ped Derm 20:323–326, 2003;
AD 132:797–800, 1996; JAAD 10:600–610, 1984
Pachydermoperiostosis (Touraine–Solente–Gole syndrome)
JAAD 31:941–953, 1994; AD 124:1831–1834, 1988
Panhypopituitarism
Ghatan p.165, 2002, Second Edition
Pinta – tertiary (late phase) – hypohidrosis and atrophy with
thinning and wrinkling of skin overlying large joints
Rook
p.1274, 1998, Sixth Edition
Poland’s syndrome
Scand J Plast Reconstr Surg 20:313–318,
1986
Porokeratotic eccrine ostial and dermal duct nevus – resemble
nevus comedonicus; linear keratotic papules with central
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