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Lipoid proteinosis: A review with two case reports
Vishal Kabre1, Smitha Rani1, Keerthilatha M Pai1
1Department of Oral Medicine and Radiology, Manipal College of Dental Sciences, Manipal University, Manipal, Karnataka, India.
Contemporary Clinical Dentistry
|June 23, 2015
Summary
Lipoid proteinosis (LP) is a rare genetic disorder causing hyaline material deposition. This report details two oral manifestation cases of LP, highlighting its varied presentation.
Area of Science:
- Dermatology
- Genetics
- Oral Medicine
Background:
- Lipoid proteinosis (LP) is an exceptionally rare autosomal recessive genodermatosis.
- It involves the deposition of amorphous hyaline material in various organs, notably the skin and upper aerodigestive tract mucous membranes.
Observation:
- This paper presents two classic cases of Lipoid proteinosis exhibiting significant oral manifestations.
- Neither case involved a reported history of consanguinity, which is often associated with recessive genetic disorders.
Findings:
- The oral cavity is identified as the most extensively affected area in patients with Lipoid proteinosis.
- The cases underscore the diverse clinical presentations of LP, even in the absence of consanguinity.
Implications:
- Understanding the oral manifestations of Lipoid proteinosis is crucial for timely diagnosis and management.
- Further research into the genetic and clinical spectrum of LP can improve patient outcomes.
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