Oral manifestations of lipoid proteinosis in a 10-year-old female: a case report and literature update

Publication date: Available online 14 February 2018
Source:Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology
Author(s): Kevin C. Lee, Scott M. Peters, Yen Chen Kevin Ko, Timothy C. Kunkle, Michael A. Perrino, Angela J. Yoon, Elizabeth M. Philipone
Lipoid proteinosis (LP) is a rare autosomal recessive disorder characterized by the deposition of amorphous hyaline material in the dermis and submucosal connective tissue. Herein, we present a case of LP with significant oral, dermatological, and neurological manifestations occurring in a 10 year-old Asian female. In addition to the more typical oral findings of restricted tongue movement and labial and buccal mucosal involvement, this case highlights an unusual pattern of gingival enlargement infrequently reported in the literature. As LP almost always involves the oral cavity, often before the onset of cutaneous lesions, it is important for dental providers to be familiar with the oral manifestations of this disease. Early detection and diagnosis of LP by the dental practitioner can help expedite proper multidisciplinary care and lead to significant reductions in patient morbidity and mortality.



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