Clinical, Biological and Therapeutic Features of a Patient with Acro Dermato Ungual Lacrimal Tooth Syndrome

Background: Acro-Dermato-Ungual-Lacrimal-Tooth syndrome is a rare autosomal dominant disorder resulting from TP63 gene mutation. ADULT syndrome is clinically manifested by ectodermal dysplasia, limb defects, nasolacrimal duct obstruction, absence of orofacial clefting, primary hypodontia and the early loss of permanent teeth. The aim of this report was to study the clinical, histological and therapeutic features of the early loss of permanent teeth of a 22-year-old Tunisian female. Case Presentation: A 22-year-old female, born to clinically healthy and consanguineous parents, was referred to the removable partial denture department for full mouth rehabilitation. The patient's medical history included a continuous follow up of her chronic dacryocystitis and a hand surgery at the age of 17. On general examination, the patient was mildly mentally retarded, underweight and photosensitive. She presented a fine hair, hypoplastic mammary glands and bilateral nasolacrimal duct obstruction complicated with corneal abscess. The examination of her digits showed onychodysplasia, fingers camptodactyly and toes syndactyly. The oral examination showed the presence of a temporary tooth and the early loss of several permanent teeth. The present teeth were peg shaped and mostly decayed. Enamel was poorly formed. Temporary and decayed permanent teeth were extracted. Polarized light microscopy was then performed to observe half teeth sections stained with hematoxylin-eosin. The other half was observed under scanning electron microscopy. Results: The patient underwent full mouth rehabilitation with a complete fixed maxillary prosthesis and a combined mandibular prosthesis with extracoronal castable precision attachment. The histological findings revealed that enamel was hypoplastic, hypocalcified and dystrophic. The acellular extrinsic fiber cementum and vertical periodontal fibers were absent. The cellular intrinsic fiber cementum was present on the root layer and on a great part of the enamel surface. Conclusion: The premature loss of permanent teeth reported in ADULT syndrome patients can be the consequent result of the absence the periodontal attachment.