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.

Collagen metabolism and basement membrane formation in cultures of mouse mammary epithelial cells. Induction of 'assembly' on fibrillar type I collagen substrata.

Collagen metabolism was compared in cultures of mouse mammary epithelial cells maintained on plastic or fibrillar type I collagen gel substrata. The accumulation of dialysable and non-dialysable [3H]hydroxyproline and the identification of the collagens produced suggest no difference between substrata in the all over rates of collagen synthesis and degradation. The proportion of the [3H]collagen which accumulates in the monolayers of cultures on collagen, however, markedly exceeds that of cultures on plastic. Cultures on collagen deposit a sheet-like layer of extracellular matrix materials on the surface of the collagen fibres. Immunoprecipitation of the labelled extracts, electrophoresis, indirect immunofluorescence and immunoperoxidase techniques reveal the presence of type IV collagen, along with laminin and heparan sulfate proteoglycan in this layer, in excess over the amounts detectable on cells cultured on plastic. Transformed cells on collagen produce and accumulate more [3H]collagen, yet are less effective in basement membrane formation than normal cells, indicating that the accumulation of collagen alone and the effect of interstitial collagen thereupon do not suffice. Thus, exogenous fibrillar collagen appears to enhance, but is not sufficient for proper assembly of collagenous basement membrane components near the basal epithelial cell surface.

[Histiocytofibroma and Dupuytren's palmar fibromatosis. Comparative autohistoradiographic study of their proliferative and biosynthetic activities]. / Histiocytofibrome et fibromatose palmaire de Dupuytren. Etude autohistoradiographique comparative de leurs activités prolifératives et biosynthétiques.

The proliferative rate and synthesis of proteins was studied in histiocytofibromas and in nodules of Dupuytren's palmar fibromatosis. Incorporations of tritiated thymidine and tritiated proline were used and revealed by autohistoradiography. In the two diseases, proliferation and protein synthesis occurred predominantly in cells of the vascular walls while the other cells constitutive of the lesions appeared less active.

Immunohistologic and ultrastructural study of the sclerotic skin in chronic graft-versus-host disease in man.

Thirteen skin biopsies were performed on 8 patients at different stages of skin sclerosis in chronic graft-versus-host disease (GVHD). On the same skin biopsies an immunostaining with antibodies directed against Types I and III procollagen, Types I, III, IV, V collagen, and laminin, and an ultrastructural study were performed. Alterations were observed at the dermal-epidermal junction and in the superficial dermis with a large deposit on Type III procollagen in the incipient scleroses and of Type I procollagen in the oldest ones. In this sclerotic superficial dermis, collagen fibers of irregular diameter were associated with mast cells and active fibroblasts, macrophages, and lymphocytes in close contact. The skin sclerosis in chronic GVHD might be considered a form of cutaneous fibrosis with features of excessive tissue repair related to an immunologic reaction between lymphocytes of the graft and tissue host cells.

Immunohistochemical localization of laminin, type IV and type V collagen in basal cell carcinoma.

Laminin, type IV and type V collagen were localized in twenty-five basal cell carcinomas (BCCs) by immunofluorescence. All basal cell carcinomas were separated from the connective tissue by a continuous basement membrane (BM) which always contained the three antigenic determinants. Nodular and cystic BCCs, but not pagetoid BCCs, contain linear and globular deposits of these BM components. The linear deposits probably represent BM debris that remains undigested and the globular deposits correspond to residual cytoplasmic fragments occurring during apoptosis. These results suggest that BM components are synthesized by epithelial cells and are secreted to polymerize at the dermo-epidermal junction.

Distribution of laminin, a basement membrane glycoprotein in epithelial proliferations. A preliminary study in the breast, the lungs and uterine cervix.

Distribution of laminin, a basement membrane glycoprotein, was studied by an indirect immunofluorescence technique in benign, dysplastic and malignant lesions of breast, lungs and uterine cervix as compared with normal tissues. Laminin was present with a linear and continuous staining in epithelial and vascular basement membranes in normal tissues, benign and dysplastic lesions and in in situ carcinomas of the uterine cervix. By contrast, laminin was absent or very irregularly distributed around malignant infiltrating tumoral clusters. Of particular interest was the linear and disrupted labeling observed in intra-epithelial carcinomas of breast and bronchi. Loss of laminin in these conditions could be related to a potential tumoral invasion.

Episodic progression and regression of basal cell carcinomas.

Metabolic activity and structure of the stroma adjacent to basel cell carcinomas are closely related to cytological aspects and proliferative activity of the neoplastic cells. Basal cell carcinomas without clear evidence for regression compress or infiltrate an apparently normal dermis where cellular replication is low. Conversely regressing zones of basal cell carcinomas are surrounded by a remodelled stroma where fibroblasts and endothelial cells show signs of proliferation. When regression affects the palisaded array of cells, clefts occur between the basal cell carcinoma and the adjacent stroma. Progression of most types of basal carcinomas may proceed by successive extension and partial regression. More than one type of stroma reaction may be present ina single basal cell carcinoma and the overall pattern may be regarded as the result of successive phases of growth and regression of the neoplasm.