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Unusual oral findings in dermatosparaxis (Ehlers–Danlos syndrome type VIIC)
Author(s) -
De Coster P.J.,
Malfait F.,
Martens L.C.,
De Paepe A.
Publication year - 2003
Publication title -
journal of oral pathology and medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.887
H-Index - 83
eISSN - 1600-0714
pISSN - 0904-2512
DOI - 10.1034/j.1600-0714.2003.00170.x
Subject(s) - medicine , ehlers–danlos syndrome , hypodontia , dentistry , dermatology
A 13‐year‐old patient with dermatosparaxis (Ehlers–Danlos syndrome type VIIC), an autosomal recessive disorder of procollagen‐I‐N‐proteinase, is presented. The oral findings comprise micrognathia, hypodontia, localized microdontia, opalescent tooth discoloration, root dysplasia, pulp obliteration, severe gingival hyperplasia, frontal open bite, and severe restriction of TMJ mobility. The reported anomalies suggest the need for expanding the present phenotypic spectrum. This is the first report on oral findings in the syndrome.