Lipoid proteinosis is a rare autosomal recessive multisystem disorder caused by mutations in the extracellular matrix protein 1 gene. It is characterized by infiltration of amorphous hyaline in the dermis and submucosal connective tissue. We report the case of 36-year-old woman with history of hoarseness since infancy and multiple episodes of right parotitis. Dermatological examination found bead-like papules on the margins of the eyelids, thickened skin lesions on the elbows and knees, enlargement of the tongue with dental impressions and lingual frenulum infiltration. Skin biopsy showed histopathological findings of lipoid proteinosis. The patient was treated by acitretin for 6?months with no improvement of the skin lesions. Three months later, patient presented a left parotitis with a cutaneous fistula through which abscess was discharged. The objective of the present report is to describe this rare entity and report that lipoid proteinosis may be associated with recurrent parotitis caused by infiltration and stenosis of Stensens duct.

Author Name: Chiguer Meriem , Ouadi Zahra , Amal Said and Hocar Ouafa

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Keywords: Urbach-Wiethe Disease Lipoid Proteinosis Parotitis

ISSN: 2320-5407

EISSN: 2320-5407

EOI/DOI: 10.21474/IJAR01/10288

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