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Results for “Skin Abnormalities”

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At least 145 records · Page 8Linked to original sources
PubMed1987

Allergy to suxamethonium: persisting abnormalities in skin tests, specific IgE antibodies and leucocyte histamine release.

Twenty-one patients, who had previously experienced an anaphylactic reaction to suxamethonium during general anaesthesia, were selected for this study. Initially, skin tests with muscle relaxants were carried out in the twenty-one patients, detection of specific anti-choline IgE in nineteen, and leucocyte histamine release in seventeen. These three tests were then repeated between 1 year and 4 years after the initial evaluation. In the majority of patients, sensitization to the muscle relaxants persisted for more than 1 year after the anaphylactic reaction. Only three patients out of twenty-one (4%) had negative skin tests when retested 1-4 years later. A reduction in leucocyte histamine release was noticed in one of the seventeen retested patients (6%). Modifications of anti-choline IgE were observed in five of nineteen patients (26%). The persistence of sensitization to suxamethonium may result from repeated stimulation by occasional contacts with quaternary ammonium compounds. This study demonstrates the reliability of skin tests, leucocyte histamine release and detection of anti-choline IgE to diagnose allergic reactions to suxamethonium, even when they are performed a long time after the initial anaphylactic reaction.

Alcuronium↗
PubMed1986

Amyotrophic lateral sclerosis: histologic, histochemical, and ultrastructural abnormalities of skin.

We studied the reticular dermis in patients with amyotrophic lateral sclerosis (ALS) and controls with or without neurologic diseases. By light microscopy, collagen bundles in ALS dermis were reduced in amount and more loosely woven than in controls. Electronmicroscopy revealed a significant negative correlation between duration of illness and the diameter of collagen fibrils in patients with ALS. There was also a marked increase of amorphous material positive for ruthenium red in the ground substance. These findings were not observed in controls.

Adult↗
PubMed2005

Acute gastroenteritis in children.

BACKGROUND: Gastroenteritis (GE) is inflammation of the mucous membranes of the gastrointestinal tract, and is characterised by vomiting and/or diarrhoea. The most common causes are viruses, but bacterial, protozoal and helminthic GE occur, particularly in developing countries. Vomiting and diarrhoea can be nonspecific symptoms in children, and the diagnosis of viral GE should be made after careful exclusion of other causes. OBJECTIVE: This article outlines the assessment and management of children with acute GE. DISCUSSION: The most important complication of GE is dehydration. The amount of weight loss as a percentage of normal body weight provides the best estimate of degree of dehydration. Clinical signs are not present until the child has lost at least 4% of their body weight. The best signs for identifying dehydration include decreased peripheral perfusion, abnormal skin turgor, and an abnormal respiratory pattern. Fluid replacement is the mainstay of management and most infants and children can be rehydrated safely with oral rehydration solution. Antiemetics and antidiarrhoeals are not indicated in children with acute GE.

Acute Disease↗
PubMed1989

Focal dermal hypoplasia. Current concepts and differential diagnosis.

Focal dermal hypoplasia syndrome (FDH) is a congenital disorder of mesodermal derivation with some ectodermal participation. The disorder, first described in 1962, is characterized by tenacious defects consisting of thinning of the skin; herniations of adipose tissue; abnormal skin pigmentation and other skeletal, dental, ocular, hair, and nail abnormalities. A rare instance of focal dermal hypoplasia syndrome is reported as characterized by papillary gingival hyperplasia. Differential diagnostic guidelines that separate focal dermal hypoplasia from a host of other skin and mucous membrane disorders are discussed along with therapeutic modalities and the possible relationship of human papillomavirus to focal dermal hypoplasia syndrome.

Adult↗
PubMedDate not supplied

Dyskeratosis congenita: unusual presenting features within a kindred.

We report on a family in which one member is affected by dyskeratosis congenita (DC), who had two cousins who died at 44 months and 36 months, respectively, with aplastic anemia and neurological abnormalities. The patient affected by DC presented with bone marrow hypoplasia at age 4; DC was diagnosed at age 6. In DC the marrow abnormalities rarely appear before the skin manifestations. The observation of this kindred poses the question whether an extremely early onset with a rapidly fatal course before the appearance of skin abnormalities is possible. We believe this report to be important for the differential diagnosis of DC and other forms of congenital aplastic anemia.

Anemia, Aplastic↗
PubMed1991

Acrylamide neuropathy in a South African factory: an epidemiologic investigation.

Acrylamide monomer concentrations and peripheral neurotoxicity were investigated in workers at a factory producing polymer. Monomer levels ranged from 0.07 to 2.5 times the recommended exposure limit of the U.S. National Institute for Occupational Safety and Health (1988). Those workers with exposure exceeding the limit had increased prevalences of acrylamide-related abnormalities. Logistic regression showed dose-response relationships with exposure for symptoms, abnormal sensation, decreased motor strength, abnormal gait or rombergism, and skin abnormalities. The overall prevalence of acrylamide-related abnormality was 32% in the total work force and 67% among those exceeding the limit compared with 14% in those exposed below the limit. Environmental exposure determinations and conventional clinical approaches are relatively insensitive measures. Associations might have been stronger if a biological exposure measure had existed and if quantitative neurometric techniques had been applied. Electrophysiological methods are not readily available in many settings; the use of the Vibration II for quantitative field testing holds promise.

Acrylamides↗
PubMed1989

Ehlers-Danlos syndrome type VI: clinical manifestations of collagen lysyl hydroxylase deficiency.

We reviewed the clinical findings in 10 patients with lysyl hydroxylase deficiency (Ehlers-Danlos syndrome type VI) and report here the range of clinical severity in these patients. The distinctive feature common to all patients was muscle hypotonia with joint laxity in the newborn period, and moderate to severe kyphoscoliosis either was present or developed in almost all patients. Most patients also had some degree of skin abnormality observed in other types of Ehlers-Danlos syndrome: bruisability, abnormal scarring, and soft, distensible skin. These patients also are at risk for potentially catastrophic arterial rupture.

Body Height↗
PubMed1993

Reflex sympathetic dystrophy in children: an orthopedic perspective.

To design diagnostic criteria for reflex sympathetic dystrophy (RSD) and to initiate a prospective treatment protocol, we reviewed our experience with 49 episodes of RSD in 36 children. There were 24 females and 12 males; mean age at diagnosis was 13.4 years (range: 8 to 19); mean time from pain onset to correct diagnosis was 9.2 months (range: 1 to 53). Lower extremity involvement predominated. Pain was "severe" in 61%, and skin color changes, swelling, hyperesthesia, abnormal skin temperatures, muscle weakness, and decreased range of motion were all present in at least 75% of cases. Osteopenia was observed in 15 of 38 radiographs; of 24 bone scans, 7 were normal, 11 showed increased uptake, and 6 demonstrated decreased uptake. Of the 23 children who had psychological evaluations, 83% revealed some type of significant emotional dysfunction. Analgesic and antiinflammatory medications were not helpful, nor were local injections or regional blockades effective. An inpatient diagnostic and rehabilitation program for treating chronic pain, including orthopedics, rheumatology, psychology, and twice-daily physical therapy was most likely to lead to resumption of age-appropriate activities. Despite extensive physiological testing, physician, parent, and/or patient reluctance to accept absence of a primary organic disease was common. We present diagnostic criteria for pediatric RSD.

Adolescent↗
PubMed1997

Multiple, juxtasutural, cranial hyperostoses and cardiac tumor: a new hamartomatous syndrome?

We report on a Japanese girl with multiple cranial hyperostoses and a cardiac tumor, both of which manifested in early childhood. Unique juxtasutural lesions characterized the cranial findings, including a chain of almost symmetrical osseous protuberances involving the frontozygomatic and frontoparietal junctions, and discrete bony bumps on the right occipitoparietal junction and left temporo-occipital junction. These lesions histologically consisted of thickened mature bone intervened with sparse fibrous tissues, mimicking osteoma. The cardiac mass remained pathologically unknown, but was shown to have fatty elements on magnetic resonance imaging (MRI). The patient showed no evidence of gnathic hyperostoses, ophthalmological abnormalities, skin lesions, or other visceral abnormalities, which ultimately precluded known hamartomatous syndromes with craniofacial hyperostoses, such as Gardner and Proteus syndromes. Yet regional Proteus syndrome could not be completely excluded. The craniofacial deformity as a sequel of hyperostoses in our patient superficially resembled that of X-linked calvarial hyperostosis; however, the vacuolated histiocytes that characterized the hyperostotic lesions were not found in our patient. The present disorder may represent a hitherto unknown hamartomatous syndrome.

Bone Neoplasms↗
PubMed1998

Hyperlipidemia and cutaneous abnormalities in transgenic mice overexpressing human apolipoprotein C1.

Transgenic mice were generated with different levels of human apolipoprotein C1 (APOC1) expression in liver and skin. At 2 mo of age, serum levels of cholesterol, triglycerides (TG), and FFA were strongly elevated in APOC1 transgenic mice compared with wild-type mice. These elevated levels of serum cholesterol and TG were due mainly to an accumulation of VLDL particles in the circulation. In addition to hyperlipidemia, APOC1 transgenic mice developed dry and scaly skin with loss of hair, dependent on the amount of APOC1 expression in the skin. Since these skin abnormalities appeared in two independent founder lines, a mutation related to the specific insertion site of the human APOC1 gene as the cause for the phenotype can be excluded. Histopathological analysis of high expressor APOC1 transgenic mice revealed a disorder of the skin consisting of epidermal hyperplasia and hyperkeratosis, and atrophic sebaceous glands lacking sebum. In line with these results, epidermal lipid analysis showed that the relative amounts of the sebum components TG and wax diesters in the epidermis of high expressor APOC1 transgenic mice were reduced by 60 and 45%, respectively. In addition to atrophic sebaceous glands, the meibomian glands were also found to be severely atrophic in APOC1 transgenic mice. High expressor APOC1 transgenic mice also exhibited diminished abdominal adipose tissue stores (a 60% decrease compared with wild-type mice) and a complete deficiency of subcutaneous fat. These results indicate that, in addition to the previously reported inhibitory role of apoC1 on hepatic remnant uptake, overexpression of apoC1 affects lipid synthesis in the sebaceous gland and/or epidermis as well as adipose tissue formation. These APOC1 transgenic mice may serve as an interesting in vivo model for the investigation of lipid homeostasis in the skin.

Animals↗
PubMed2005

[Urologic findings in pseudoxanthoma elasticum: report one case].

CASE REPORT: A 21-year-old woman presented with retinal angioid streaks and yellowish streak skin abnormalities in neck and axillary folds. Skin biopsy showed bluish-gray tangled masses of calcified elastic fibers in the mid- to lower dermis suggestive of Pseudoxanthoma elasticum (PXE). She consulted in Urology Department for lower urinary tract infection. Renal ultrasonography revealed multiple highly reflective foci in the corticomedullary junction. COMMENT: Pseudoxanthoma elasticum is a rare genetic disorder characterised by fragmentation and calcification of elastic fibers in the skin and media of arteries. Frequent manifestations include hypertension, angina pectoris, transient cerebral ischemic attacks, intermittent claudication, upper gastrointestinal bleeding, retinal angioid streaks and thickened skin. A characteristic appearance of highly reflective foci in the renal parenchyma have been reported in patients with PXE, but it's not specific for this syndrome. However, the presence of this structural pattern in a young patient with dermatological abnormalities should lead to the consideration of PXE in the differential diagnosis list. Besides ultrasonography findings, only a ureteral disruption case in a patient underwent ureteroscopic manipulation has been described to our knowledge. In our case, the true significance of these disease in recurrent urinary tract infection is uncertain.

Adult↗