Lipoid proteinosis is an inherited disorder characterized histologically by the deposition of an amorphous eosinophilic material in skin and other tissue. Histochemical demonstration of lipid in the deposits led early investigators to suspect that the disorder was a lipoidosis. However, recent biochemical and electron microscopical studies have suggested that the material is principally a glycoprotein elaborated by fibrocytes.
Fibroblast cultures were established with the use of tissue from a cutaneous lesion of a woman with this disorder to determine whether an error of lipid metabolism occurred in the disease. An assay of fibroblast lipids revealed normal quantities of all lipid fractions. This appears to confirm that the accumulation of lipids in the lesions of this disease is not due to a primary metabolic defect, but is a secondary phenomenon, probably due to the affinity between lipoproteins and glycoproteins.
Shore RN, Howard BV, Howard WJ, Shelley WB. Lipoid Proteinosis: Demonstration of Normal Lipid Metabolism in Cultured Cells. Arch Dermatol. 1974;110(4):591–594. doi:10.1001/archderm.1974.01630100051012
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