Lipoid proteninosis:case report

Abstract

URBACH-WIETHE is a very rare autosomally recessive disorder characterized by infiltration of hyaline material into skin,oral cavity,larynx,and internal organs.in this report we persent a case of a 22 year old woman with small scars on her face,hoarseness,firm tongue and beaded papules along the margins of eyelids,who was referred to the deparment of ophthalmology of Kerman university No3: hospital for ophthalmological consultation.pathological examinations of the biopsy specimens from eyelid lesions,confirmed the case as an URBACH-WIETHE disease.