Sunburn and swelling: the edema of erythema.
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It is unknown whether T cells infiltrating the pretibial skin of patients with thyroid-related pretibial dermopathy represent a primary immune response or participate in a nonspecific inflammatory process. To characterize these T cells at the molecular level, we examined the T-cell antigen receptor variable region gene usage in pretibial skin biopsy specimens obtained from patients with early and late stages of pretibial dermopathy and from individuals with unrelated inflammatory conditions of the pretibial skin. RNA extracted from pretibial biopsy specimens and peripheral blood lymphocytes was reverse transcribed and amplified with the polymerase chain reaction and 22 V alpha and 23 V beta gene-specific oligonucleotide primers. The resulting T-cell receptor (TcR) V alpha and V beta transcripts were verified by Southern hybridization analysis using TcR C-region-specific, digoxigenin-labeled oligonucleotide probes. Compared with matched peripheral blood lymphocytes, the pretibial TcR V alpha and V beta gene repertoire expressed was heterogeneous but revealed marked restriction of V alpha and V beta gene usage in samples derived from patients with active inflammatory pretibial dermopathy of recent onset. In contrast, greater diversity of the TcR V alpha gene repertoire and loss of TcR V beta gene restriction were noted in patients with long-standing, clinically inactive pretibial dermopathy. TcR V gene usage in pretibial tissue and peripheral blood lymphocyte samples obtained from control subjects was unrestricted. Limited variability of TcR V gene usage in early pretibial dermopathy may reflect a primary immune response of antigen-specific T lymphocytes infiltrating the pretibial skin in thyroid-related pretibial dermopathy.
Skin fibroblasts from the shoulder and lower extremities of normal individuals, as well as from patients with pretibial myxedema (PTM) were grown in culture. When cells reached the monolayer stage, they were labeled with 3H-glucosamine and tested for hyaluronic acid synthesis in the presence of either serum from PTM patients or normal human serum. All the fibroblasts from the pretibial area synthesized 2 to 3 times more hyaluronic acid when incubated with PTM sera than when incubated in normal human serum. Fibroblasts cultured from skin of the back or prepuce did not respond to PTM sera. This heat-stable, protease-sensitive, and dialyzable, fibroblast-stimulating factor is not a 7S gamma-globulin. The enhanced sensitivity to PTM sera exhibited by fibroblasts from the lower extremities may explain why the lesions in this disease are restricted primarily to that area.
Pretibial epidermolysis bullosa is a rare form of dominant dystrophic epidermolysis bullosa. The disease was diagnosed after considerable delay in a large Belgian family and was remarkable for its late age at onset and its misleading clinical presentation in the proband, which strongly resembled keratosis lichenoides chronica. Both recessively and dominantly inherited forms of dystrophic epidermolysis bullosa have been shown to be linked to the collagen type VII gene, COL7A1. Two-point linkage analysis with two intragenic polymorphisms (PvuII, AluI) in COL7A1 was performed. Strong genetic linkage between the disease in this family and COL7A1 was demonstrated by a lod score of 4.45 (theta = 0) for the AluI polymorphism. The observed intrafamilial variability of clinical phenotypes contradicts the presently proposed classification of dominantly inherited dystrophic epidermolysis bullosa.
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The term 'baboon syndrome' (BS) was introduced 20 years ago to classify patients in whom a specific skin eruption resembling the red gluteal area of baboons occurred after systemic exposure to contact allergens. Thereafter, similar eruptions have been reported after systemic exposure to beta-lactam antibiotics and other drugs. In addition to the presentation of 2 of our own cases, we have reviewed and characterized the main clinical and histological aspects of published reports of drug-related baboon syndrome (DRBS) and compared the primary clinical signs from such cases to those found in other distinct drug eruptions. Of approximately 100 published baboon syndrome cases, 50 were identified as drug-induced. Of these, 8 were representatives of systemically induced contact dermatitis (SCD), and 42 were examples of drug eruptions elicited by systemic administration of either oral or intravenous drugs. The main clinical findings included a sharply defined symmetrical erythema of the gluteal area and in the flexural or intertriginous folds without any systemic symptoms and signs. 14 of 42 cases were elicited by amoxicillin, 30 of the 42 patients were male, and latency periods were between a few hours and a few days after exposure. DRBS is a rare, prognostically benign and often underdiagnosed drug eruption with distinct clinical features. The term baboon syndrome, however, does not reflect the complete range of symptoms and signs and is ethically and culturally problematic. Moreover, baboon syndrome is historically often equated with a mercury-induced exanthem in patients with previous contact sensitization. Symmetrical drug-related intertriginous and flexural exanthema, or SDRIFE, specifically refers to the distinctive clinical pattern of this drug eruption, and the following diagnostic criteria are proposed: 1) exposure to a systemically administered drug either at the first or repeated dose (excluding contact allergens); 2) sharply demarcated erythema of the gluteal/perianal area and/or V-shaped erythema of the inguinal/perigenital area; 3) involvement of at least one other intertriginous/flexural localization; 4) symmetry of affected areas; and 5) absence of systemic symptoms and signs.
Ethylbutylthiourea (EBTU) is an accelerator used in the production of chloroprene (neoprene) rubber. EBTU occurs in a mixture with diethylthiourea (DETU) and dibutylthiourea (DBTU) in the accelerator. An analytical method originally developed for analysis of zinc dithiocarbamates in rubber has been used to analyse EBTU, DETU and DBTU in a knee brace responsible for an allergic contact dermatitis in a gardener suffering from arthrosis. EBTU was isolated and gave positive reactions when tested as a pure compound. The test reaction was accompanied by positive reactions to DETU and DBTU.
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An earlier report described cutaneous lesions, consisting of erythema with or without necrosis, on the legs and/or feet of elderly diabetics and the cause was suggested to be an altered reaction to precipitating factors such as cardiac decompensation. The present investigation concerns the cutaneous reactions to traumatization with local heat or cold to the skin of legs and forearms of 35 diabetics and 25 controls. Petechiae within the area of traumatization with either heat or cold were observed more often in diabetics than in controls. They occurred more frequently on the legs than on the forearms. Among the controls, petechiae were observed only in those over 50 years of age and only on the legs. In the diabetics under 50, petechiae were almost always observed when the duration of diabetes was 10 years or more but seldom in young patients with diabetes of short duration. The duration of diabetes was not significantly related to the occurrence of petechiae in diabetics over 50. In these diabetics, moreover, petechiae developed after traumatization with heat of a lower temperature than that which caused petechiae to appear in corresponding controls. The initial skin lesions in dermopathia diabetica (Melin) have a reddened border. The skin of the legs of some of the diabetics developed an intensely reddened border round the area of experimental heat or cold traumatization. These patients were either elderly diabetics or younger patients with diabetes of long duration. Each of them had dermopathia diabetica and each developed atrophic circumscribed skin lesions on the site of traumatization. Nineteen diabetics had dermopathia diabetica and 16 of them developed atrophic circumscribed skin lesions on the site of traumatization, lesions which were never seen in the controls. Thus, diabetics differ from controls in their reaction to a certain thermal trauma. The possible reasons for this altered reaction are discussed.
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Histopathological findings from eight cases of xerosis vulgaris of the leg (XVL) were compared to those from nine normal controls using light microscopy and one micron epoxy resin-embedded sections stained with toluidine blue and basic fuchsin (T-F) and electron microscopy with ultra-thin sections. Three sub-layers were identified in the stratum corneum of all cases of XVL and normal controls by differences in color in T-F stained specimens and by differences in density in electron microscopic findings. Electron microscopically, the intermediate sub-layer within the three-layered structure showed an uneven density with many empty spaces in both normal controls and cases of XVL. Statistically, only the intermediate sub-layers of XVL increased significantly in number (p less than 0.05) as well as in thickness (p less than 0.001), compared to normal controls. These findings suggest that the intermediate sub-layers play an important role in holding water in the skin.
Three cases of pigmented basal cell carcinoma were reported which needed to be differentiated from malignant melanoma based on their location and clinical features. Clinicopathologically, they were characterized by a large number of melanophages in the stroma, blockade melanocytes in the parenchyma, and phagocytosis of melanosomes in tumor cells as well as a large number of intraparenchymal melanophages. Two cases were characterized by the aggregation of melanophages in cystic spaces caused by stellate atrophy.
We describe here a case of EMO syndrome, which is defined as a combination of exophthalmos, pretibial or localized myxedema, and hypertrophic osteoarthropathy. A 34-year-old Japanese man with Graves' disease developed the characteristic eye changes. He showed 22 mm protrusion of both eyes and hypertrophy of the right lateral rectus muscle as well as both superior rectus muscles by computerized tomography. He subsequently developed lightly erythematous, indurated, nonpitting, peau d'orange plaques and nodules on his lower legs. Finally, he developed Graves' acropathy with hypertrophic osteoarthropathy in the metacarpal bones. Histological examination of myxedematous skin showed typical deposition of mucin accompanied by changes in the amount and distribution of elastic fibers. In addition, the lesional skin showed localized hyperhidrosis, a rarely reported complication of pretibial myxedema. We speculate that this hyperhidrosis of the lesional skin was brought about by stimulation of peripheral sympathetic nerves by surrounding mucin deposition, in the setting of poorly controlled hyperthyroidism.