Lipoid Proteinosis: A Case Report

Dr. Neeta Pradhan; Dr. Rahul Mahadik; Dr. Rajesh Karekar; Dr. Shreepad; Dr Sanjay

Volume : III, Issue : X, October - 2013

Lipoid Proteinosis: A Case Report

Dr. Neeta Pradhan, Dr. Rahul Mahadik, Dr. Rajesh Karekar, Dr. Shreepad, Dr Sanjay

Abstract :

Lipoid proteinosis (LP), also known as Urbach Weithe disease, is a rare disorder with autosomal recessive inheritance, resulting in progressive widespread deposition of Periodic– Acid Schiff (PAS) positive hyaline material in the skin, mucous memanes of the upper respiratory tract and other internal organs. Classical clinical presentation includes acneiform pox–like skin scarring with formation of yellowish, waxy papules, beaded eyelid papules and laryngeal infiltration leading to hoarseness of voice. Neurological affection in the form of seizures and behavioural disturbances with associated bean–shaped calcifications in the temporal and hippocampal areas may be present. We hereby report a young male with a classical presentation of Lipoid proteinosis.