A clinicopathologic study of six cases of lipoid proteinosis

We have reviewed the clinical and histological presentations of six cases of lipoid proteinosis. Deposits in the dermis were heterogeneous in nature and location. In the first stage of the disease's evolution, basement membrane components accumulate at the normal site of vascular, muscular, and epithelial basement membranes. Later on, lipids and amorphous material fill the superficial dermis, pushing apart the normal collagen bundles. Such deposits seem to be organized around preserved reticulin fibers. The amorphous material did not react with antibodies to collagen types I, III, IV, and V.