Ectodermal dysplasia syndrome

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Ectodermal dysplasia syndrome

ORPHA:79373Category

Also called Ectodermal dysplasia

What it is

The term ''ectodermal dysplasia'' defines a heterogeneous group of heritable disorders of the skin and its appendages characterized by the defective development of two or more ectodermal derivatives, including hair, teeth, nails, sweat glands and their modified structures (i.e. ceruminous, mammary and ciliary glands). The spectrum of clinical manifestations is wide and may include additional manifestations from other ectodermal, mesodermal and endodermal structures.

Key facts

Prevalence
6-9 / 10 000
Age of onset
Infancy, Neonatal
Classified as
Category

Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.

Genes reported in subtypes

BRAFCDH1CTNNB1CTNND1DSPEVCEVC2GJA1IFT122IFT43IFT52IRF6KRASKRT14LSSMAP2K1MAP2K2NECTIN1NECTIN4PEX1PEX6PIGLPKP1PRKD1RIPK4RMRPROGDISLC13A5TWIST2WDR19WDR35

Orphanet records these genes on 20 more specific entries under this disorder, not on this entry itself:

A well-studied disease is usually split into subtypes, and the gene is curated on whichever one the evidence belongs to. Open a subtype to see which gene goes with it.

Cross-references

GARD 6317MEDDRA 10010452MESH D004476MONDO 0019287MONDO 19287UMLS C0013575

Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.

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