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Definition

A form of ectoderma dysplasia, a heterogeneous group of disorders due to abnormal development of two or more ectodermal structures. Characterized by absence of sweat glands, sparse scalp hair, rare conical teeth and immunological abnormalities resulting in severe infectious diseases.

Acronym

EDAID1

Synonyms

Ectodermal dysplasia, anhidrotic, with immunodeficiency X-linked
Ectodermal dysplasia anhidrotic with immune deficiency
Ectodermal dysplasia hypohidrotic with immunodeficiency
EDA-ID
HED-ID
Hyper-IgM immunodeficiency X-linked with ectodermal dysplasia hypohidrotic
NEMO deficiency
XHM-ED

Keywords

Ectodermal dysplasia

 
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