1/6. Livedoid vasculopathy in a patient with factor V mutation (Leiden).BACKGROUND: Frequently, no underlying disease can be detected in patients with livedoid vasculopathy. For these forms, an unknown vaso-occlusive or thrombogenic process has been accused to play a role. Thus, a patient with livedoid vasculopathy was examined for different parameters which can be involved in coagulopathies. methods: Laboratory studies for different autoantigen reactive immunoglobulins, cryoglobulins, and circulating immune complexes were carried out. Besides dermatopathologic examination, a biopsy specimen was analyzed by direct immunofluorescence for immunoglobulin (Ig) and complement deposits. Furthermore, hemostaseological function tests including activated protein C (APC) resistance were undertaken. RESULTs: Positive only at very low titres were antinuclear antibodies and c-ANCA, all other parameters were within normal ranges or negative. Direct immunofluorescence revealed IgM, C3 and fibrogen deposits. Hemostaseological function tests demonstrated a pathologic activated protein c resistance and PCR analysis a heterozygous defect of the factor V (Leiden). CONCLUSIONS: The diagnosis of livedoid vasculopathy associated with factor V mutation (Leiden) was made. Since the underlying cause for livedoid vasculopathy often remains unknown, we suggest that hemostaseological function tests including APC resistance and factor V gene mutation analysis should be carried out. Further studies have to follow in order to elucidate the role of mutant factor V in livedoid vasculopathy and in cutaneous ulcerations.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
2/6. Immunohistochemical characterization of the 'intimal proliferation' phenomenon in Sneddon's syndrome and essential thrombocythaemia.Cellular changes were immunocytochemically characterized in skin vessels of five patients with idiopathic generalized racemose livedo (Sneddon's syndrome), and one patient with localized racemose livedo associated with essential thrombocythaemia. antibodies against alpha-smooth muscle-actin, tropomyosin, desmin, vimentin, factor viii-related antigen, human endothelial cells (CD31), human macrophages (CD68), and HLA-DR positive cells (CR3/43) were used. Conventional light microscopy showed, in all cases, intimal thickening of ascending arteries and arterioles as a result of an accumulation of cells and extracellular hyalinized material. None of the specimens showed infiltration with polymorphonuclear leucocytes or macrophages. The cells in the region of the intimal hyperplasia showed intense positive immunostaining for alpha-smooth muscle actin and tropomyosin. Staining for the intermediate filament desmin was localized to the resident smooth muscle cells of the media, whereas staining for vimentin was found in all types of cells in both the intima and media. Positive immunostaining for factor viii-related antigen and CD31 was strictly confined to the endothelial cells lining the narrowed lumina of the vessels. No positive staining with either antibody was observed in totally occluded vessels. Cells in the subintimal space did not show reactivity for CD68 in any of the specimens, but two cases showed solitary cells with positive staining for HLA-DR in this region. There were no differences in staining pattern between Sneddon's syndrome and essential thrombocythaemia with any of the antibodies. Our results support the assumption that the 'intimal proliferation' in both diseases is caused by colonization of the subendothelial space with contractile cells of possible smooth muscle origin.(ABSTRACT TRUNCATED AT 250 WORDS)- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
3/6. Study of antiphospholipid antibodies in a patient with Sneddon's syndrome and her family.BACKGROUND: Sneddon's syndrome is a disease characterized by livedo reticularis and cerebrovascular lesions, with a hereditary transmission and unknown etiopathogenesis. A number of reports have documented a link between antiphospholipid antibodies and Sneddon's syndrome with different results. The present work was designed to sequentially study antiphospholipid antibodies in a patient with Sneddon's syndrome and her family and their potential role in thrombotic events. We used cardiolipin and a mixture of phospholipids from rabbit brain as antigen for antiphospholipid assays to determine diagnostic usefulness. CASE DESCRIPTIONS: A patient with Sneddon's syndrome and 12 available family members belonging to three generations were evaluated to determine the presence of antiphospholipid antibodies (lupus anticoagulant and anticardiolipin antibodies) during vascular thrombotic events and asymptomatic periods. CONCLUSIONS: Our results support a temporal relation between thrombotic events in Sneddon's syndrome and lupus anticoagulant; anticardiolipin antibodies remained invariable. Our index case patient and her father could be diagnosed as having primary antiphospholipid antibody syndrome. aspirin was not effective in preventing thrombosis. After the onset of oral anticoagulant therapy, no recurrences were seen. The use of a mixture of phospholipids as antigen could present some advantages in serological studies performed in antiphospholipid syndromes.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
4/6. A reticulate vascular abnormality in patients with lymphoedema: observations in eight patients.We describe eight patients with lymphoedema who had prominent compressible ridges of tissue in a reticulate pattern, situated predominantly on the upper part of the lower leg. In five patients the lymphoedema was primary, two patients had circumferential venous ulceration, and one had marked venous disease with a small ulcer. One patient had a squamous cell carcinoma of the medial thigh and dysplastic keratoses in the distribution of the reticulate ridges. In three of the four cases in whom histological examination of the ridges was performed, the skin at these areas was demonstrated to contain grossly dilated angular vessels in the mid-dermis, many with valves visible. The vessel walls had a single layer of endothelial cells (anti-factor viii-related antigen positive) and a basement membrane containing type IV collagen. Abnormal elastic tissue in these biopsies was similar to that in erythema ab igne. Indirect lymphography in one case did not demonstrate dilated lymphatic vessels. The body site distribution and clinical pattern of the abnormality appeared to be similar to erythema ab igne but associated with an underlying abnormality of lymphatic rather than blood vasculature. We propose that our cases may represent 'lymphoedema ag igne'.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
5/6. Plantar thrombotic nodules with marked neovascularization in diabetes: a symptom which has been overlooked?A 69-year-old woman with diabetes has had multiple subcutaneous nodules on the soles for 11 months. She suffered from uncontrolled diabetes mellitus, hypercholesteremia, and cardiovascular thrombosis and had a past history of photocoagulation therapy for proliferative diabetic retinopathy. Histological examination revealed vascular thrombosis surrounded by neovascularization in the subcutaneous tissue. The basement membranes of vessels were thickened and strongly stained with periodic acid-Schiff. An increased serum concentration of von willebrand factor antigen was observed. With control of her diabetes, the nodules almost disappeared. Her curious skin manifestation is speculated to be a diabetes-mellitus-related change in which endothelial injury and the subsequent induction of angiogenetic factors may play important roles. It is possible that this kind of skin manifestation has been overlooked or not examined histopathologically.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
6/6. Intravascular large cell lymphoma: a patient with asymptomatic purpuric patches and a chronic clinical course.Intravascular large cell lymphoma (malignant angioendotheliomatosis) is a rare, multifocal, intravascular neoplasm of lymphoid cells that preferentially involves the vasculature of the skin and central nervous system. We describe a 54-year-old man with asymptomatic purpuric patches on the lower extremities for 10 years duration and a more recent lesion on the right arm. A biopsy specimen showed intravascular collections of tumor cells with irregular nuclear contours and prominent nucleoli. These cells were leukocyte common antigen (CD45), CD20, and CDW75 positive, but CD3, CD43, CD45RO, and cytokeratin negative. polymerase chain reaction analysis of the skin for immunoglobulin heavy chain gene rearrangement detected a clonal population of B cells, supporting the diagnosis of a B-cell lymphoma. Peripheral blood showed no abnormal circulating cells. This case of malignant angioendotheliomatosis is unusual for its prolonged clinical course and presence of purpuric patches.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |