[MIM 103 285]

Acronym for "Acro -Dermato-Ungueal-Lacrimal-Tooth".

(Propping-Zerres syndrome)

Very rare: < 1/106. Autosomal dominant transmission with incomplete penetrance and variable expressivity of a mutation of the TP63 gene (3q 27). It presents many similarities with the EEC syndrome (see this term). 

Association of:

- ectrodactyly of hands and/or feet

- freckles in excessive quantities

- onychodysplasy

- obstruction of the tear ducts

- hypodontia or early loss of permanent teeth.

Sometimes abnormalities of the sweat glands with risk of hyperthermia.

 Anesthetic implications: 

fragile teeth, monitoring of temperature

References : 

Updated:  December 2019