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Article
April 1939

XANTHOMATOSIS: IV. LIPOID PROTEINOSIS (PHOSPHATIDE LIPOIDOSIS)

Author Affiliations

ROCHESTER, MINN.
From the Section on Dermatology and Syphilology (Dr. Montgomery) and the Section on Laryngology, Oral and Plastic Surgery (Dr. Havens) of the Mayo Clinic.

Arch Otolaryngol. 1939;29(4):650-661. doi:10.1001/archotol.1939.00650050698006
Abstract

There are various types of xanthoma which may result in lesions involving the mucous membranes of the mouth, throat, larynx and trachea. We wish to report a case of lipoid proteinosis (Urbach1) in which such lesions were presented and to demonstrate that this condition is a definite clinical and especially a definite histologic and histochemical entity. It will be differentiated especially from other forms of xanthomatosis involving the mucous membranes, such as disseminate xanthomatosis, hepatosplenomegaly with lipoidosis of the skin and mucous membrane of Bürger and Grütz, and in some cases Hand-Schüller-Christian disease.2

Lipoid proteinosis was first recognized as a form of xanthomatosis by Urbach3 and by Urbach and Wiethe in 1929,4 under the title "lipoidosis cutis et mucosae," although it had been described by Siebenmann5 in 1908 and by Wiethe6 in 1924 and in 1926 as a hyaline change of the mucous membranes associated with hyperkeratotic lesions of the

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