ADULT SYNDROME: DENTAL FEATURES OF A VERY RARE CONDITION

L. AZZI, V. MAURINO, R. VINCI, F. CROVERI, A. BOGGIO, A. TAGLIABUE, J. SILVESTRE-RANGIL, L. TETTAMANTI

Article ID: 6577
Vol 31, Issue 2S1, 2017
DOI: https://doi.org/10.54517/jbrha6577
Received: 9 July 2017; Accepted: 9 July 2017; Available online: 9 July 2017; Issue release: 9 July 2017

Abstract

The Acro-Dermato-Ungual-Lacrimal-Tooth syndrome (ADULT syndrome) is one of the rarest ectodermal dysplasias and it is associated with several malformations involving especially the limbs. The most clinical features are the presence of ectrodactyly, syndactyly, hypermelanosis or multiple lentigines, onhycodysplasia, abnormalities in the lacrimal duct, recurrent conjuntivitis, photophobia, mammarian hypoplasia, hypotrichosis and frontal alopecia, hypohydrosis, cutaneous photosensitivity, nasal bridge prominence, exfoliative dermatitis and xerosis. The ectodermal dysfunction expresses itself with conoid teeth, enamel hypoplasia, dentinal dysplasia and especially hypodontia, with following functional and aesthetic defects. We report the case of an 11-year-old Caucasian girl affected by ADULT syndrome.


Keywords

ADULT syndrome;ectodermal dysplasia;dental agenesis;dental malformation;rare disease


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Supporting Agencies



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