Biomedical subjects
Publications and source records attributed to L S Ostlere.
We describe four patients in whom porokeratosis coexisted with lympoedema of the legs. A possible pathogenetic link between the two disorders is discussed, as well as the therapeutic implications and the novel physical sign of lymphoedema bulging through the porokeratotic lesions.
We have studied patients who have positive-patch test reactions to fragrance-allergic screening substances fragrance mix (FM) or Myroxylon pereirae resin (balsam of Peru) for immediate contact reactions to the standard FM, the constituents of the FM and Myroxylon pereirae resin. In the fragrance-positive subjects (n = 60), there were positive immediate contact reactions to Myroxylon pereirae resin in 56.6% and to FM in 11.6%. In a control group (n = 50) of eczematous, patch test-negative patients there were positive immediate reactions to Myroxylon pereirae resin in 58.0% subjects and to FM in 12.0%. The absence of a significant difference between the fragrance-allergic group and control group is in keeping with a non-immunological basis for the majority of the immediate reactions seen.
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OBJECTIVE: Previous endocrine studies of women with acne have produced diverse results. This study was designed to seek evidence, from endocrine and genetic studies, for impaired steroid biosynthesis in patients with acne. DESIGN: Adrenal stimulation tests with synthetic adrenocorticotrophic hormone (ACTH) were performed. MEASUREMENTS: Steroid hormones were measured basally and 30 minutes after ACTH. The results were correlated with analysis of the steroid 21-hydroxylase gene (CYP21). PATIENTS: Fifty-one consecutive female patients (mean age 27.1 years) referred with acne. RESULTS: The median plasma 17-hydroxyprogesterone (17-OHP) before and 30 minutes after ACTH were 2.5 nmol/l (range 1.1-8.2) and 7.3 (2.1-17.8) nmol/l which were significantly above normal female controls (n = 11, mean age 25.6 years) at 1.5 (0.9-4.2) and 4.6 (2.6-8.4) nmol/l. Eighteen of 51 acne patients showed an abnormal 17-OHP response. The 21-hydroxylase gene (CYP21) was examined for major deletions and for three common point mutations in 31 of the patients (14 with exaggerated 17-OHP response). One patient had a deletion of CYP21 on one allele consistent with carrier status for the classical congenital adrenal hyperplasia (CAH). Five patients, one of whom had a normal 17-OHP response to Synacthen, were heterozygous for the val 281 leu mutation in exon 7 of the CYP21 and were therefore carriers for a mutation associated with late-onset CAH. One patient with a raised 17-OHP response was homozygous for the splice site mutation in intron 2 and one patient with a normal 17-OHP response was heterozygous for the mutation. None of the patients had the ile 172 asn mutation. Eight of the 31 acne patients who had CYP21 gene analysis were carriers for mutations in the 21-hydroxylase gene but only six would have been detected by an abnormal response of 17-OHP on stimulation. CONCLUSION: Although alterations of the CYP21 gene were more common in acne than in controls there is a poor correlation between these events and raised steroids and acne. Factors other than mild impairment of CYP21 contribute to the variability of the clinical phenotype in hyperandrogenic states including acne.
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Substance P has various immunomodulatory effects, including in vitro modification of lymphocyte proliferation and cytokine release. Elevated levels of substance P and increased staining of substance P-positive nerve fibres have been reported in atopic dermatitis patients. We examined fluoresceinated substance P binding to a range of lymphocyte subsets and compared the results in atopic dermatitis, non-atopic psoriasis patients and normal controls. Fluoresceinated substance P and phycoerythrin-labelled monoclonal antibodies to CD3, CD4, CD8, CD57, CD19 and CD14 were incubated in duplicate with Ficoll-Hypaque separated peripheral blood mononuclear leukocytes. With flow cytometry the fluoresceinated substance P-positive cells were identifiable as a peak of positively fluorescent cells, and the percentages of positive cells were measured. We have demonstrated binding of fluoresceinated substance P to all subsets examined, with significantly less binding to atopic dermatitis CD3-, CD8- and CD57-positive cells. This may affect cytokine release and hence be important in the pathogenesis of atopic dermatitis.
We report the case of a female infant with an intrauterine herpes simplex type II infection in zosteriform distribution. She was treated with several courses of intravenous acyclovir leading to healing of the skin with segmental scarring. This patient is unusual in that the infection occurred in zosteriform distribution without any evidence of systemic involvement.
The neuropeptides substance P (SP) and vasoactive intestinal peptide (VIP) are present in the nerve endings in the skin and SP is thought to be present at abnormal concentrations in atopic dermatitis (AD) patients. Th1 and Th2 imbalance in AD has been the focus of recent immunological investigations and a preferential Th2 response by atopic cells on stimulation has been proposed. We wished to establish whether neuropeptides acted on T cells to affect their cytokine profile directly, using an accessory cell-independent stimulus (anti-CD3 monoclonal antibody and neuropeptides at several concentrations. We found that interferon (IFN)-gamma and interleukin (IL)-4 release were lower in AD. SP had an enhancing effect on both IFN-gamma and IL-4 at physiological concentrations (10(-10)-10(-6) mol/L) in AD, which was significantly different from controls (P < 0.05). VIP had inhibitory effects over this range in AD and in controls. We conclude that these neuropeptides have a modest effect on T-cell cytokine release and that their action is not cytokine-specific.
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BACKGROUND: In many studies, an increase in total cholesterol and triglycerides with isotretinoin therapy have been shown and investigators have commented on potential cardiovascular risk. A low intake of linoleic acid, the main essential fatty acid in man, may act as an independent risk factor for coronary heart disease. In vitro etretin alters both the incorporation of extracellular fatty acids into cell membranes and the fatty acid composition of the cell membrane itself. It is, therefore, important to establish whether isotetinoin has any effect on the metabolism of polyunsaturated fatty acids. METHODS: The effect of treatment with isotretinoin for 4 months on the metabolism of polyunsaturated fatty acids in patients with acne was assessed. Quantitative total cholesterol and triglycerides as well as plasma phospholipid, triglycerides, and cholesteryl ester fatty acids were measured in 12 patients and red cell phosphatidylethanolamine, phosphatidylcholine, and phosphatidylinositol fatty acids were measured in 13 patients before and after isotretinoin therapy. RESULTS: There was a significant increase in the concentrations of cholesterol (P < 0.02) and triglycerides (P < 0.04) during treatment. There was no significant difference is plasma phospholipids, triglycerides, and cholesterol esters, or in the red cell phosphatidylethanolamine, phosphatidylcholine, and phosphatidylinositol during isotretinoin therapy. CONCLUSIONS: This study failed to demonstrate any effect of isotretinon on the metabolism of polyunsaturated fatty acids. There was a significant increase in total cholesterol and triglyceride levels following isotretinoin therapy supporting the findings of many previous studies.
BACKGROUND: Seborrheic dermatitis (SD) is a frequent complication of infection with the human immunodeficiency virus (HIV). Most studies examining the cause of SD have concentrated on the roles of Pityrosporum ovale and sebaceous lipids. Previous studies of skin surface lipid from patients with SD have produced conflicting results, with some authors reporting an abnormal lipid composition and others finding little or no abnormality. METHODS: The composition of skin surface lipid was studied in 15 HIV-positive and 10 HIV-negative men with SD, in 14 HIV-positive men without SD, and in 16 unaffected controls. Total lipids were extracted from unaffected forehead skin into petroleum ether and separated into lipid classes by thin layer chromatography. The lipid classes were quantitated by densitometry after charring with sulfuric acid. RESULTS: Patients, HIV-positive with SD, had significantly lower proportions of free fatty acid (FFA) and higher levels of triglyceride than normal controls. Patients, HIV-positive without SD, had a significantly increased proportion of FFA compared to HIV-positive patients with SD. Patients with SD, both HIV-positive and HIV-negative, had a similar pattern of skin surface lipid. Levels of FFA were lower and those of triglyceride higher than in the patients unaffected by SD, whether HIV-positive or not. There was no significant difference found between groups in free cholesterol, wax esters, and squalene. CONCLUSIONS: Abnormalities of skin surface lipid composition may play a part in the development of SD in both HIV-positive and HIV-negative men.
We report a large kindred in which a punctate palmoplantar keratoderma (PPK) is associated with malignancy, including Hodgkin's disease, renal, breast, pancreatic and colonic adenocarcinomas. The family was traced through four generations, and over 320 individuals were identified, of whom 49 had punctate PPK. The punctate PPK appeared to be inherited as an autosomal dominant trait with variable penetrance. Ten of the 43 adults (23%) with punctate PPK developed malignancies, and five of these developed before the age of 50. Of the 271 unaffected individuals, six (2%) have developed malignancies, one prior to the age of 50. The association of keratoderma and malignancy is discussed.
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