ImmunoDeficiency Resource (IDR)
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Autosomal dominant anhidrotic ectodermal dysplasia and T-cell immunodeficiency
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Printable factfile
Patients present impaired development of skin appendices, resulting in sparse hair, conical teeth, and anhidrosis/hypohidrosis. Host defense is also impaired and the patients can have multiple and severe bacterial disease.
Diagnosis
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ESID/PAGID recommendations by IDR
Additional Information
Incontinentia pigmenti, The doctor's doctor
Nemo Gene
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eMedicine
Combined B-cell and T-cell disorders, eMedicine
Research programs, clinical trials
European Initiative for Primary Immunodeficiencies, coord. Edvart Smith
Human Pigmentation Disorder Linked to Genetic Defect in Inflammatory Pathway, National Institute of Environmental Health Sciences, USA
Genetic and biochemical analysis of the NF-kB signalling pathway, Courtois G. et al, France