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An Official Publication of the Indian Association of Oral and Maxillofacial Pathologists

CASE REPORT Table of Contents   
Year : 2009  |  Volume : 13  |  Issue : 2  |  Page : 81-84
Lipoid proteinosis

Department of Oral Pathology, M. S. Ramaiah Dental College, Bangalore, Karnataka, India

Correspondence Address:
Roopa Shamsundar Rao
Department of Oral Pathology, M.S. Ramaiah Dental College and Hospital, New Bel Road, MSR nagar, Bangalore
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0973-029X.57675

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Lipoid proteinosis is a rare disorder with autosomal recessive inheritance, characterized by progressive deposition of hyaline material in the skin, mucous membrane, and different organs of the body, resulting in a multitude of clinical manifestations. A 62-year-old male presented with hoarseness of voice since infancy, eyelid beading, and generalized acneiform scars on the facial skin and extremities, and yellowish papules on his tongue and buccal mucose. The patient was diagnosed clinically as a case of Lipoid proteinosis, which was confirmed by skin and mucosal biopsy. The objective of the present work is to describe this rare entity, with approximately 250 cases found in medical literature. This case report also illustrates that Lipoid proteinosis may show protean clinical features and yet may remain undiagnosed for many years. This report will hopefully spawn further studies that will lead to early diagnosis.

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Journal of Oral and Maxillofacial Pathology | Published by Wolters Kluwer - Medknow
Online since 15th Aug, 2007