Visceral involvement in lipoid proteinosis

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Abstract
The literature about lipoid proteinosis has previously documented the presence of a peculiar, characteristic, and ill-defined chemical substance in certain inexplicable patterns of deposition in the dermis of the skin, submucosa of the mouth, tongue, tonsil, larynx, pharynx, trachea, esophagus, stomach, rectum, and vagina, and also in the testes, eye, and brain. Six cases were studied, four of them previously unreported. Cutaneous and visceral biopsies from three of these patients, plus autopsy specimens from another show that the llpoglycoprotein that characterizes the disease is also present in very small amounts outlining occasional blood vessels in the jejunum, urinary bladder, appendix, pancreas, lung, kidney, lymph node, and striated muscle. Lipoid proteinosis is unquestionably a generalized disorder involving many organs besides the skin and mucous membranes.