NUMERO: #20260607

Título: FOURTH CASE OF REGIONAL ODONTODYSPLASIA IN EPIDERMAL NEVUS SYNDROME - CHANCE OR ASSOCIATION?

Nome do Apresentador: Sudarat KIAT-AMNUAY

Categoria do Trabalho: Pôster - Relato de Caso ou Série de Casos

Área Temática: Áreas afins no caráter multidisciplinar

Resumo: Background. The exceptionally rare somatic mosaicism, epidermal nevus syndrome (ENS) is especially interesting because its mutations are post-zygotic, occurring after conception, causing localized or tissue-specific alterations. One of the most unique of associated tooth-related phenomena is regional odontodysplasia (RO). We present the fourth such case. Case Description. A 25-year-old man with a large, irregular congenital hyperkeratotic/melanotic plaque from his right spine to his sternum had been long-diagnosed with ENS and now sought treatment for irregular and aching right maxillary teeth. The teeth showed mottled discoloration and irregular, sometimes vertical enamel hypoplasia, with portions of enamel obviously fractured. Routine radiographs did not show irregular pulpal calcifications or a "ghost-like" appearance, but CBCT and microscopy showed irregularities in pulpal shapes, with globular calcifications in the pulp. Diagnosis & Conclusions. The diagnosis was RO. Since ENS and RO are both regional in nature, we suggest that RO can be genetically associated with ENS.

Autor 1:  Sudarat KIAT-AMNUAY

E-mail 1:  [email protected]

Autor 2:   Heather L. Athon

E-mail 2: 

Autor 3 :  Jerry E. Bouquot

E-mail 3:  [email protected]