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December 1958

Lipoid Proteinosis

Author Affiliations

Departments of Dermatology, Children's Memorial Hospital, and the University of Illinois College of Medicine (Drs. Falk and Medansky); Departments of Medicine, Children's Memorial Hospital and Northwestern University College of Medicine (Dr. Tracy); Department of Pediatrics, Research and Educational Hospitals, University of Illinois (Dr. Sholler).

AMA Am J Dis Child. 1958;96(6):727-729. doi:10.1001/archpedi.1958.02060060729014

Although lipoid proteinosis is observed primarily in the child, references to this condition in the pediatric literature are scant. The term lipoid proteinosis was first used by Urbach4 in 1932. This, however, was the renaming of this group of cases, as they had been previously called lipoidosis cutis et mucosae.2 In 1924 Wiethe1 had reported the first of these cases as a disease consisting of skin and mucous membrane involvement, but not until 1929 did Urbach and Wiethe3 show their cases to be identical. Their patients showed a hoarseness present almost since birth, with growths involving the mucous membranes and vocal cords. Nodules and plaques occurred on the gums, mucous membranes, floor of the mouth, soft palate, frenulum of the tongue, and uvula. Mucous membranes of the genital and rectal areas were similarly affected. Incidental findings reported by other observers included alopecia of the scalp, loss

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