LIPOID PROTEINOSIS OF THE SMALL-BOWEL

We describe a 65-year-old-man who presented with acute gastrointestina l bleeding secondary to massive submucosal deposits of hyaline materia l in the small bowel. The histochemical and ultrastructural features o f the hyaline substance were typical of lipoid proteinosis, a rare cut aneous disorder in which, to our knowledge, symptomatic compromise of internal organs has not been described previously. The patient was lat er found to have mild but characteristic mucocutaneous lesions of lipo id proteinosis, as well as asymptomatic deposits in other gastrointest inal sites. Our case documents that severe visceral involvement may oc cur in lipoid proteinosis, even in previously undiagnosed patients wit h mild cutaneous manifestations of the disease.