1/5. Small bowel schwannoma with diffuse subcutaneous lipomatosis. Case report and literature review.A case of a small-bowel schwannoma with diffuse familiar lipomatosis is described. This case underlines the rarity of the neoplasm and its probably chance association with subcutaneous lipomatosis. The intestinal neoplasm was diagnosed preoperatively by upper gastrointestinal endoscopy and a small-bowel enema; computed tomography scan confirmed the intestinal lesion. attention is focused on the morphological features of intestinal schwannomas and their biological behaviour.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
2/5. Restrictive dermopathy and fetal behaviour.We report three siblings from consecutive pregnancies affected with restrictive dermopathy (RD). During the second pregnancy, fetal behavioural development and growth were studied extensively using ultrasound at 1-4 week intervals. Dramatic and sudden changes occurred in fetal body movements and growth but not until the end of the second trimester of pregnancy. Prominent at that time were prolonged periods of fetal quiescence and very low heart rate variability, together with abnormally executed body movements of short duration. Retarded femoral development and jerky abrupt fetal body movements (abnormal movement quality) were already present in the early second trimester of pregnancy. Facial anomalies emerged despite the presence of fetal mouth movements. The clinical features of RD were only partly explained by present knowledge of skin development and the fetal akinesia deformation sequence hypothesis. Quantitative assessment of fetal movements proved to be a poor early marker for antenatal diagnosis of this disorder.- - - - - - - - - - ranking = 5keywords = behaviour (Clic here for more details about this article) |
3/5. Post-transplant T-cell lymphoproliferative disorder/T-cell lymphoma: a report of three cases of T-anaplastic large-cell lymphoma with cutaneous presentation and a review of the literature.AIMS: To report the clinical, pathological and immunohistochemical features of three cases of post-transplant T-cell lymphoproliferative disorder (T-PTLD) T-cell lymphoma with primary cutaneous presentation. methods AND RESULTS: Three cases of primary cutaneous post-transplantation anaplastic large-cell lymphomas occurred in renal transplant recipients and were shown to display a T-cell immunophenotype; all were ALK 1 protein and EMA negative and two were Epstein-Barr virus positive using in-situ hybridization. Two displayed a CD4 phenotype, two were focally CD56 and all three were negative for the cytolytic enzyme granzyme B. In two cases monoclonality was established by T-cell receptor gene rearrangement study. All presented with nodular cutaneous involvement and all were ultimately fatal. CONCLUSION: T-PTLDs are uncommon histological subtypes both in a general context and associated with cutaneous presentation. Our findings suggest clinicopathological and immunophenotypic similarities to primary cutaneous anaplastic large-cell lymphoma but with a progressive clinical behaviour similar to previously reported T-PTLD and to systemic nodal ALK- anaplastic large-cell lymphoma.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
4/5. Thermal transition studies of a mature xanthelasma by differential scanning calorimetry.A large xanthelasma which had been present for at least 5 years was removed surgically from a normolipaemic female age 54 years, and examined in the fresh state by differential scanning calorimetry. Thermal transitions recorded over the range 30-40 degrees C suggest that the lipid present, predominantly esterified cholesterol, is not bound to protein or other tissue components, and that the chronicity of mature xanthelasmata as with ectopic lipid deposits at other sites is enhanced by chemical modification of lipid and effects on component phase behaviour, which are significant at local skin temperature.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
5/5. Adrenoleucodystrophy: dermatological findings and skin surface lipid study.X-linked adrenoleucodystrophy (ALD) is a rare disorder of the very-long-chain fatty acid (VLCFA) metabolism. Cutaneous findings observed in a 29-year-old man included patchy non-cicatricial alopecia of the scalp, intense oily seborrhoea of the head, mild dry-scaling ichthyosis-like appearance of the trunk and legs and pseudo-acanthosis nigricans of the folds. Scanning electron-microscopic examination of the scalp hair showed trichorrhexis-nodosa-like fractures and several structural anomalies of the hair shaft. The skin surface lipid profile showed a marked increase in lignoceric acid (C24:0) which comprised over 60% of the fatty acid detectable in the fractions of triglycerides, diglycerides and free fatty acids. This behaviour of the VLCFA in the surface lipids corresponds to that observed in the plasma. If this finding were confirmed, the investigation of surface lipid composition could represent a useful non-invasive technique for the study of ALD.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |