Basan syndrome

Synonym(s)

Ectodermal dysplasia absent der-matoglyphics

Definition

Autosomal dominant syndrome consisting of ectodermal dysplasia, absent derma-toglyphic pattern, nail abnormalities, and a simian crease

Pathogenesis

Inherited; mutation site unknown

88 Bather's itch

Clinical manifestation

Thin skin; simian crease; multiple dental caries; absent or decreased eyebrows; nail dystrophy; sparse or absent scalp hair; decreased sweating; photophobia; absent dermatoglyphic pattern

Differential diagnosis

Anhidrotic ectodermal dysplasia; hidrotic ectodermal dysplasia; focal dermal hypoplasia; Down's syndrome; progeria

Therapy

No effective therapy References

Masse JF, Perusse R (1994) Ectodermal dysplasia. Archives of Disease in Childhood 71(1)11-2

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