title: Oral findings in a child with lipoid proteinosis: a case report and review.
abstract: Lipoid proteinosis, a very rare autosomal recessive genodermatosis, results in hyaline material deposition in the skin and mucous membrane of various organs leading to multisystem involvement. A case report of a 12-year-old female child is presented here who showed classic features of the disease with generalized thickening, hardening, and scarring of the skin and vocal cord infiltration causing voice changes. The patient also had numerous oral mucosal and dental findings. The knowledge of the clinical features of the disease may help the oral health professional in rendering the appropriate treatment in order to improve the quality of life deteriorated by the disease.
authors.affiliation: Department of Pedodontics and Preventive Dentistry, MCODS - affiliated to Manipal University, Manipal, India. drsneedhamainali@gmail.com
authors.forename: S
authors.initials: S
authors.lastname: Mainali
authors.forename: R
authors.initials: R
authors.lastname: Nayak
authors.forename: S
authors.initials: S
authors.lastname: Gaur
mesh_terms: D000848:Anodontia / Q000175:diagnosis; D002648:Child; D003241:Consanguinity; D003731:Dental Caries / Q000175:diagnosis; D005260:Female; D006801:Humans; D007180:Incisor / Q000002:abnormalities; D008065:Lipoid Proteinosis of Urbach and Wiethe / Q000175:diagnosis*; D009059:Mouth Diseases / Q000175:diagnosis*; D014059:Tongue / Q000002:abnormalities; D014076:Tooth Diseases / Q000175:diagnosis*; D014987:Xerostomia / Q000175:diagnosis