Lipoid Proteinosis: Two Case Reports Including Liver Biopsies, Special Blood Lipid Analyses, and Treatment With a Lipotropic Agent

Case Reports1 July 1954LIPOID PROTEINOSIS: TWO CASE REPORTS INCLUDING LIVER BIOPSIES, SPECIAL BLOOD LIPID ANALYSES, AND TREATMENT WITH A LIPOTROPIC AGENTJAMES TOMPKINS, M.D., IRWIN M. WEINSTEIN, M.D.JAMES TOMPKINS, M.D.Search for more papers by this author, IRWIN M. WEINSTEIN, M.D.Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-41-1-163 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptLipoid proteinosis is a peculiar abnormality of fat deposition characterized by the appearance of white or yellow plaques and nodules in the skin and mucous membranes, producing hoarseness due to vocal cord involvement, sometimes associated with diabetes mellitus, a history of consanguinity in the parentage and familial occurrence. The disease is quite rare, only 34 cases having been reported in the literature to date. The course is apparently benign unless respiratory difficulty ensues. Wiethe1published the first notable description of this condition in 1924, but Urbach2deserves credit for recognizing the nature of the disease and for coining the name...Bibliography1. Wiethe C: Kongenitale diffuse Hyalinablagerungen in den oberon Luftwegen familiär Auftretend, Ztschr. f. Hals-, Nasen- u. Ohrenh. 10: 359-362, 1924. Google Scholar2. Urbach E: Ueber eine familiäre Lipoidose und der Haut und Schleimhäute auf Grundlage einer diabetischen Stoffwechselstörung, Arch. f. Dermat. u. Syph. 157: 451-466, 1929. CrossrefGoogle Scholar3. Ramos e Silva S: Lipoid proteinosis, Arch. Dermat. and Syph. 47: 30, 1943. Google Scholar4. PriceLaRosaSettle HWVEB: Lipoidosis cutis et mucosae, Arch. Dermat. and Syph. 55: 42, 1947. CrossrefGoogle Scholar5. WileSnow UIDS: Lipoid proteinosis: report of a case, Arch. Dermat. and Syph. 43: 134, 1941. CrossrefGoogle Scholar6. DragstedtVon ProhaskaHarnes LRJHP: Observations on a substance in pancreas which permits survival and prevents liver damage in depancreatized dogs, Am. J. Physiol. 117: 175, 1936. CrossrefGoogle Scholar7. LewisPage LAIH: Electrophoretic and ultracentrifugal analysis of serum lipoproteins of normal, nephrotic and hypertensive persons, Circulation 7: 707, 1953. CrossrefMedlineGoogle Scholar This content is PDF only. To continue reading please click on the PDF icon. Author, Article, and Disclosure InformationAffiliations: Chicago, Illinois*Received for publication December 4, 1953.From the Sections of Dermatology and Hematology, Department of Medicine, and the Argonne Cancer Research Hospital, University of Chicago, Chicago, Illinois.†Lipocaic is the name suggested by Dragstedt and his associates6 for the active principle in the pancreas which prevents or relieves the fatty infiltration of the liver developing in insulin-treated depancreatized dogs and which makes possible the long survival of these animals in a good nutritive state. Preparations of lipocaic by the methods originally described were not available, so recourse has been made to a pancreas powder prepared by removing the fat from beef pancreas by acetone or gasoline. This defatted pancreas has approximately one fifth the activity of the original extracts of Dragstedt, and has been administered orally in capsules or tablets containing 0.4 gm. of the dry powderized extract to the two patients reported in this publication. PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited byHyalinosis cutis et mucosaeRadiological finding in lipoid proteinosisHyalinosis cutis et mucosaeLARYNGEAL LESIONS IN URBACH-WIETHE DISEASE (LIPOGLYCOPROTEINOSIS; LIPOID PROTEINOSIS; HYALINOSIS CUTIS ET MUCOSAE)Successful surgical treatment of lipoid proteinosisA Study of Five Cases of Lipoid ProteinosisLIPOID PROTEINOSISHistochemical Study of a Case of Lipoid Proteinosis1 1 July 1954Volume 41, Issue 1Page: 163-171KeywordsFatsHematology and oncologyHospital medicineInsulinLipidsLiverMembrane proteinsMucous membranesPancreasProteins ePublished: 1 December 2008 Issue Published: 1 July 1954 PDF downloadLoading ...