Twenty-five patients with immunologically classified B cell chronic lymphocytic leukaemia (CLL) were examined for skin lesions. Six showed evidence of cutaneous involvement, and histological examination of skin biopsies from these patients revealed perivascular lymphocytic infiltration with some diffuse involvement in three. Immunohistological analysis with a range of B and T cell specific monoclonal antibodies revealed that in all cases the infiltrate was predominantly T cell in origin and that epidermotropism in three cases was also associated with T cell components. Six control patients with macroscopically normal skin were also biopsied and these biopsies subjected to the same assessment. All were normal.
After birth dramatic decreases in cardiac malonylCoA levels result in the rapid maturation of fatty acid oxidation. We have previously demonstrated that the decrease in malonyl CoA is due to increased activity of malonyl CoA decarboxylase (MCD), and decreased activity of acetyl CoA carboxylase (ACC), enzymes which degrade and synthesize malonyl CoA, respectively. Decreased ACC activity corresponds to an increase in the activity of 5Ј-AMP activated protein kinase (AMPK), which phosphorylates and inhibits ACC. These alterations are delayed by myocardial hypertrophy. As rates of fatty acid oxidation can influence the ability of the heart to withstand an ischemic insult, we examined the expression of MCD, ACC, and AMPK in the newborn human heart. Ventricular biopsies were obtained from infants undergoing cardiac surgery. Immunoblot analysis showed a positive correlation between MCD expression and age. In contrast, a negative correlation in both ACC and AMPK expression and age was observed. All ventricular samples displayed some degree of hypertrophy, however, no differences in enzyme expression were found between moderate and severe hypertrophy. This indicates that increased expression of MCD, and the decreased expression of ACC and AMPK are important regulators of the maturation of fatty acid oxidation in the newborn human heart. T he adult heart has an extremely high energy demand which is met by the oxidation of fatty acids, accounting for 60 -80% of cardiac ATP (ATP) production (1-5). Glycolysis, glucose oxidation, and lactate oxidation are responsible for the remainder of ATP production (2). Interestingly, cardiac energy substrate preference differs between the fetal and neonatal heart. In the fetal heart blood levels of fatty acids are low (6) and the heart has a low circulatory workload and meets its energy demands from glycolysis and lactate oxidation (6 -8). At birth, major cardiovascular changes occur, including a reduction in pulmonary vascular resistance and closure of the ductus arteriosus and foramen ovale. These changes are coupled to an increase in peripheral vascular resistance, and an increased workload on the newborn heart. With circulating substrate and hormonal changes there is a switch in the energy substrate preference of the heart, from glucose and lactate metabolism to fatty acid oxidation (6 -8).Although plasma fatty acid levels rapidly rise to levels seen in the adult immediately after birth (6), newborn rabbit studies have shown that the heart undergoes a transition from oxidizing glucose to oxidizing fatty acids during the 7 d immediately after birth (9,10). The time frame responsible for the maturation of fatty acid oxidation in the human newborn heart is not yet known.The increase in fatty acid oxidation after birth in the newborn rabbit heart is related to dramatic decreases in cardiac malonyl CoA levels (10,11). Malonyl CoA is a potent inhibitor of carnitine palmitoyl transferase-1 (CPT-1), the rate limiting enzyme of mitochondrial fatty acid uptake (12). Acetyl CoA Carboxy...
Summary Two unusual patients with erythema elevatum diutinum (EED) are reported. The first presented with large skin‐coloured nodules on the feet, whilst the second patient had smaller, purpuric and ulcerated nodules on the hand which contained calcium pyrophosphate crystals. This patient also had polycythaemia rubra vera and an IgA gammopathy. The wide clinical spectrum of EED is reviewed.
A case of gastro-intestinal carcinoma is reported in which extensive eczema craquele was a presenting feature.
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