J Korean Ophthalmol Soc.
1989 Aug;30(4):641-658.
Ophthalmic Findings of Urbach-Wiethe Disease
- Affiliations
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- 1Department of Ophthalmology, College of Medicine, Seoul National University, Seoul, Korea.
- 2Department of Dermatology, College of Medicine, Seoul National University, Seoul, Korea.
Abstract
- This is a rare hereditary disease characterized by hyaline and lipid deposits in the skin and mucous membrane. It starts in infancy or early childhood with hoarseness and swallowing difficulty due to the deposition of the characteristic material in the larynx and pharynx. And then, a skin eruption appears over the face and scalp with bulla, papule, crust and depressed scar forms. A pathognomonic feature is the appearance of translucent papules along free margins of eyelids, giving the appearance of "beads on a string". Other ocular involvement is rare and drusen or degeneration may be seen at the macula. We have observed two cases of Urbach-Wiethe disease in a 7-year-old girl and ll-year-old boy and confirmed by the histopathologic results.
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