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PubMed1977

Hidrotic ectodermal dysplasia: study of a large Chinese pedigree.

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 the male members. The nail and skin lesions also became severer with age. Except for the infectious eczematoid dermatitis present in the propositus, none had other skin or systemic disorders. All were relatively healthy and had normal life expectancies;

Adult↗
PubMed1997

A newly recognized autosomal dominant ectodermal dysplasia syndrome: the odonto-tricho-ungual-digital-palmar syndrome.

We report on two brothers, their mother, and 18 other relatives of five generations presenting an apparent newly recognized syndrome involving natal teeth, trichodystrophy, prominent interdigital folds, simian-like hands with transverse palmar creases, and ungual digital dystrophy, inherited as an autosomal dominant trait. In addition, the patients had hypoplasia of the first metacarpal and metatarsal bones and distal phalanges of the toes.

Adult↗
PubMed1999

Cytogenetic and molecular evidence for cutaneous mosaicism: the ectodermal origin of Blaschko lines.

The idea that skin disorders following Blaschko lines represent genetic mosaicism is widely accepted. It seems likely that the two skin types represent the two different genotypes, but this has been remarkably difficult to prove, most studies showing a mixture of cell types in biopsies from both types of skin. Only for linear epidermolytic hyperkeratosis has it been possible to show mutant cells confined to the abnormal streaks. The hypothesis proposed here to explain this paradox is that disorders following Blaschko lines are due to mutations in genes expressed in epidermal cells (keratinocytes and melanocytes) rather than in dermal fibroblasts. The work on epidermolytic hyperkeratosis used keratinocytes, whereas most studies have used skin fibroblasts. Almost all disorders following Blaschko lines are epidermal: inflammatory, dysplastic, dyskeratotic, appendage-related, or pigmentary, and the remainder can be explained on the basis of epidermal influences on the dermis. If this hypothesis is correct, it points to a useful model system for elucidating the genetic component of common dermatoses sometimes found in Blaschko lines namely eczema, psoriasis, lichen planus, and vitiligo.

Chimera↗