Open AccessOral findings in a child with lipoid proteinosis: A case report and review
Author(s) -
Sneedha Mainali,
Ramadas Nayak,
Sumit Gaur
Publication year - 2011
Publication title -
journal of the indian society of pedodontics and preventive dentistry/journal of indian society of pedodontics and preventive dentistry
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.378
H-Index - 33
eISSN - 1998-3905
pISSN - 0970-4388
DOI - 10.4103/0970-4388.79946
Subject(s) - genodermatosis , medicine , hyaline , mucous membrane , mucocutaneous zone , disease , dermatology , pathology , biochemistry , chemistry , gene
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.