1977
DOI: 10.1001/archderm.113.4.481
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Hidrotic ectodermal dysplasia: study of a large Chinese pedigree

Abstract: Hidrotic ectodermaldysplasia was found, to our knowledge, for the first time in a Chinese family in Malaysia, and it affected 15 members in five generations. The disease, which is transmitted as a non-sex-linked autosomal dominant trait, presumably originated from southern China. All 15 members had the typical nail, hair, and skin lesions, and we observed three different types of nail defects. Scalp alopeica was more extensive in the female members while keratoderma of the palms and soles was more notable in t… Show more

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Cited by 20 publications
(12 citation statements)
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“…A distinctive nail morphology as described by Jacobsen [1928] and later by Clouston [1929], Wilkey and Stevenson [1945], Gagnon et al [1989], Rajagopalan and Tay [1977], and Patel et al [19911 is the single consistent finding in all affected individuals in the families reported. Hair abnormalities (fine, brittle, or sparse) show variability between family members and all affected individuals in all families described have normal teeth and sweating.…”
Section: Resultsmentioning
confidence: 53%
“…A distinctive nail morphology as described by Jacobsen [1928] and later by Clouston [1929], Wilkey and Stevenson [1945], Gagnon et al [1989], Rajagopalan and Tay [1977], and Patel et al [19911 is the single consistent finding in all affected individuals in the families reported. Hair abnormalities (fine, brittle, or sparse) show variability between family members and all affected individuals in all families described have normal teeth and sweating.…”
Section: Resultsmentioning
confidence: 53%
“…Patient 1 ton, 1929;Wilkey and Stevenson, 1945;Rajagopalan and Tay, 1977;Hammil et al, 19881. Familial autoso-ma1 dominant sinus brachycardia has previously been documented [Sarachek and Leonard, 19721.…”
Section: Cloustonmentioning
confidence: 99%
“…Hidrotic ectodermal dysplasia, or Clouston syndrome, is an uncommon autosomal dominant genodermatosis, characterised by, among other signs, dystrophy of the nails, hyperkeratosis of the palms and soles, hypotrichosis, and presently categorised according to Freire-Maia in Group A, subgroup 1-2-3 [Clouston, 1929;Rajagopalan and Tay, 1977;Freire-Maia and Pinheiro, 19881. We report on a family with a form of hidrotic ectodermal dysplasia appearing in three consecutive generations, which we consider to be an entity separate from Clouston syndrome and categorised in Group A, subgroup 1-3 [Freire-Maia, 19771.…”
Section: Introductionmentioning
confidence: 99%
“…Nail abnormalities are usually present in all patients and may range from almost normal appearing nails to micro-or anonychia (Clouston, 1929). Nail plate changes may include thickening, brittleness, discoloration, splitting, and onycholysis (Clouston, 1929;Clouston, 1939;Rajagopalan and Tay, 1977;Hassed et al, 1996). Paronychia and nail infections are common (Clouston, 1929;Kibar et al, 1996).…”
mentioning
confidence: 99%
“…Some but not all patients develop diffuse PPK and discrete hyperpigmentations over joints, whereas strabismus and cataracts are rare (Clouston, 1929;Clouston, 1939;Hazen, 1980). Sweat function and teeth are normal (Rajagopalan and Tay, 1977;Hassed et al, 1996;Sybert, 1997). Unlike in KID syndrome, patients with Clouston syndrome lack vascularizing keratitis and SNHL.…”
mentioning
confidence: 99%