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February 1963

Lipoid Proteinosis (of Urbach-Wiethe)

Author Affiliations

Department of Ophthalmology, Hospital of the University of Pennsylvania, Department of Otolaryngology, Jefferson Medical College Hospital and Veterans Administration Hospital of Philadelphia.

Arch Ophthalmol. 1963;69(2):174-179. doi:10.1001/archopht.1963.00960040180008

Introduction  Lipoid proteinosis is a rare disease of skin and mucous membranes. Characteristic lesions of the eyelid occur, but we have found only one case in the ophthalmic literature.1 Although the earliest clinical report appeared in 1908, it was not until Urbach and Wiethe's 1929 article that the disease was extensively studied. Scattered reports have appeared since, mostly in the dermatologic and otolaryngologic literature.Various names have been given the disease. "Lipoidosis cutis et mucosae" was Urbach and Wiethe's 1929 term. Urbach renamed it "Lipoid proteinosis" in 1932 and Lundt in 1949 coined "Hyalinosis cutis et mucosae." McCusker and Caplan (1962) have suggested "Lipoglycoproteinosis."The characteristic lesions on mucous membranes and later on the skin, are small, translucent, yellowish papules. Less frequently they may appear as yellowish plaques. Hoarseness due to vocal cord involvement is the commonest complaint. Although not present at birth, lipoid proteinosis begins in infancy or

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