Oral manifestations in a boy with X-linked reticulate pigmentary disorder
نویسندگان
چکیده
Case report A 3-year-old boy was seen at the Department of Maxillo-Facial Surgery and Paediatric Dentistry of the Children’s Hospital of Trieste. The multi-systemic features of XLPDR included a number of oro-dental manifestations such as misshapen teeth, scissor bite, swallowing difficulties, agenesis of the 4 permanent second premolars, taurodontism, early resorption of deciduous roots, and premature tooth eruption in general. Other important characteristics are crowding of permanent inferior tooth crowns, severe gingivitis, enamel hypoplasia and discoloration due to multiple antimicrobial treatments.
منابع مشابه
X-linked reticulate pigmentary disorder with systemic manifestations: a new family and review of the literature.
X-linked reticulate pigmentation disorder with systemic manifestations (XLPDR) is an extremely rare genodermatosis with recessive X-linked inheritance but unknown molecular basis. In males, cutaneous involvement is characterized by reticulate hyperpigmentation of the skin that is associated with a typical facies and severe systemic involvement. In the carrier females, manifestations are apparen...
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