An infant with chronic articular and cutaneous manifestations: a new syndrome?
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Biomedical subjects
Publications and source records attributed to J I Harper.
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A woman with a 20-year history of acral pustular psoriasis of Hallopeau and recurrent pustular lesions of the forearms and lower legs, developed a B-cell lymphoma of the lip following 4 1/2 years of treatment with razoxane.
The skin of preterm infants varies considerably in its level of maturity. To understand skin absorption in premature infants better, we report a technique for the assessment of percutaneous absorption at various gestational and postnatal ages using stable, isotope-labeled (13C6) benzoic acid. Our results indicate that in the preterm infant, this method detects enhanced skin absorption in the first postnatal days, which declines over three weeks to that expected of a full-term infant. This approach also indicates an inverse relationship between gestational age and skin absorption, as well as postnatal age and skin absorption. The reported technique is a safe and noninvasive method using a model skin penetrant for the study of percutaneous absorption in preterm infants from which basic data may be derived to add to our understanding of skin barrier function.
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The dermal collagen of a patient with lipoid proteinosis was investigated by immunohistochemistry and biochemical analysis. The affected skin was found to contain significantly less collagen per unit dry weight than normal dermis but showed elevated levels of type 3 collagen with respect to type I. Purification of collagen types from affected skin after pepsin digestion showed no novel forms, but a doubling in the yield of type 5 collagen. These results correlated well with those of immunohistochemistry which showed a patchy, diffuse, widely distributed type 3 collagen and an increase in types 4 and 5 collagens associated with 'onion skin' endothelial basement membrane thickening. Estimation of collagen cross-links showed an abnormal pattern with a preponderance of the keto-imine form not normally associated with skin. These results strongly suggest that lipoid proteinosis involves a primary perturbation of collagen metabolism.
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Oral Cyclosporin A (CyA) was given for the prophylaxis of graft-versus-host disease (GVHD) to sixty-seven patients receiving a bone marrow transplant, and was found to be extremely effective in reducing the severity of acute GVHD. Side-effects of CyA included hypertrichosis, gum hypertrophy, and a rare but serious 'capillary leak' syndrome.
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