Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0036690 (sepsis)
 59,461 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Organisms with little pathogenic potential in immunocompetent hosts may produce disease in HIV-1 + patients. We describe three HIV-1 + patients in late disease who presented with pruritic papules with central ulceration over the face and arms. In all the patients the eruptions had been present for months, and the patients did not develop sepsis. Biopsy specimens in all the patients showed large Gram-positive cocci, forming tetrads. Colony morphology, catalase positivity and coagulase negativity, and resistance to nitrofurantoin were used to separate micrococci from staphylococci. Micrococcus species are usually considered normal inhabitants of the skin; however, in patients with HIV-1 disease, Micrococcus species can produce localized cutaneous infections.
Br J Dermatol 1999 Sep
PMID:Micrococcus folliculitis in HIV-1 disease. 1058 69

Acrodermatitis enteropathica is characterized by eczematous and scaly plaques on the face, scalp, acral, and anogenital regions. In addition to typical lesions, unusual prominent vesiculobullous lesions are also described. We report a full-term, 9-month-old boy who has acrodermatitis enteropathica and Pseudomonas sepsis. In this patient there were clinical findings of sepsis and eczematous vesiculobullous lesions on the periorificial and acral areas. Serum zinc level was extremely low. Pseudomonas aeruginosa was identified in cultures of blood and fluid which was aspirated from the bullous lesions. After oral zinc sulfate and intravenous antibiotic treatment his condition improved within 2 weeks.
Pediatr Dermatol
PMID:Acrodermatitis enteropathica with Pseudomonas aeruginosa sepsis. 1063 41

Epidermolysis bullosa is a group of hereditary blistering disorders for which there is no definitive therapy. Wound care is an important component of management. Regular dressing changes are required to protect blistered and eroded skin, and to prevent secondary infection and sepsis. These dressing changes can be very painful for patients with extensive erosions. We report our experience of pain management in an 11-year-old boy with severe junctional epidermolysis bullosa. Amitryptiline and cognitive behavioral techniques were effective in relieving chronic pain and discomfort. Oral midazolam 0.33 mg/kg administered 20 minutes prior to baths and dressing changes substantially improved his tolerance of wound care.
Pediatr Dermatol
PMID:Pain management of junctional epidermolysis bullosa in an 11-year-Old boy. 1063 47

Severe drug eruptions are rare, life-threatening events. The management begins with the withdrawal of the suspect drug(s). We recently confirmed that an earlier withdrawal of drugs with short elimination half-life was associated with a better survival of patients with Stevens-Johnson syndrome (SJS) or toxic epidermal necrolysis (TEN). In cases of "acute skin failure" (exfoliative dermatitis, extensive SJS or TEN), management of patients must be undertaken in specialized intensive care units or in burn units. The main principles of symptomatic therapy are the same as for major burns: warming of the environment, correction of electrolyte disturbances, high caloric intake, and prevention of sepsis. The suspected immunologic orgin of drug eruptions prompted the use of corticosteroids, immunosuppressive drugs, and anti-cytokines. Systemic corticosteroids are useful in "hypersensitivity syndrome" when visceral lesions depend on infiltration by activated cosinophils. Systemic corticosteroids were shown to be deleterious in cases of advanced TEN. Their potential usefulness at earlier stages of SJS or TEN remains controversial. High intravenous doses of cyclophosphamide or oral cyclosporin have been administered to a few patients with TEN, most often following ineffective treatment with corticosteroids for 1 to 5 days. It remains doubtful that the progression of the lesions was shortened. A few patients appeared to benefit from treatment with pentoxifyllin, a drug suppressing the production of TNF. Thalidomide, another suppressor of TNF production, significantly increased the death rate when tested in a double-blind placebo controlled trial in patients with early TEN. High dose intravenous immunoglobulins were used in 10 patients with TEN on the basis of their ability to inhibit fas-fas ligand mediated apoptosis. The potential benefit of this treatment needs confirmation by further studies. Patients and their first degree relatives should be advised to avoid the responsible drug and chemically related compounds. Regulatory agencies should be notified of such cases.
J Dermatol 1999 Nov
PMID:Treatment of severe drug eruptions. 1063 13

Toxic epidermal necrolysis is a severe, life-threatening illness with up to one-third mortality. We report a retrospective analysis of all cases treated in Royal Perth Hospital over a 20-year period from July 1978 to June 1998, by analysis of medical records. A total of 12 patients with an age range of 23-73 years was identified. The female to male ratio was 2:1, with age of onset earlier in females. All cases were associated with medications, most commonly antibiotics, anticonvulsants and allopurinol. The mortality rate was one-third (four deaths), mostly resulting from cardiorespiratory failure, renal failure and sepsis. Risk factors for death were advanced age and severe underlying disease, including diabetes, alcoholic liver disease sepsis and malignancy. Among the six patients treated with systemic corticosteroids there was only one death. Treatment with corticosteroids appeared to be beneficial, with such patients having both fewer complications and a lower mortality rate.
Australas J Dermatol 2000 Feb
PMID:Toxic epidermal necrolysis in Western Australia. 1071 97

Atopic dermatitis (AD) is frequently complicated by minor bacterial superinfections. Invasive infections such as osteomyelitis have rarely been reported. We describe two children with staphylococcal septicemia during an exacerbation of their AD. Cellulitis and underlying congenital heart disease, respectively, were considered predisposing factors for the development of bacteremia. Identical strains were isolated from the skin, and there was a significant increase in antibodies against Staphylococcus aureus capsular polysaccharide in one child. Our cases demonstrate the potential severity of bacterial skin infections in AD, especially when associated with an underlying condition that increases vulnerability to bacteremia. While their true incidence in children with AD is currently unknown, it is conceivable that systemic staphylococcal infections may be more common than previously thought. Staphylococcal bacteremia has to be considered in the differential diagnosis of fever in children with severe AD. Conversely, episodes of staphylococcal bacteremia should prompt a search for underlying predisposing factors.
Pediatr Dermatol
PMID:Staphylococcal septicemia in children with atopic dermatitis. 1079 98

Generalized atrophic benign epidermolysis bullosa (GABEB) is an autosomal recessive form of junctional epidermolysis bullosa, with milder clinical features than the Herlitz subtype. A 25-year-old man presented with the clinical and histological findings of GABEB. At the initial visit, laboratory tests revealed that he also had chronic renal failure (CRF). Usually, GABEB has a good prognosis. However, in this case, the patient had CRF as an associated complication. He died of an intracranial haemorrhage combined with sepsis after 3 weeks of hospitalization. This case suggests that renal complications can occur in this relatively mild form of epidermolysis bullosa, and may contribute to morbidity and premature mortality.
Clin Exp Dermatol 2000 May
PMID:Generalized atrophic benign epidermolysis bullosa--poor prognosis associated with chronic renal failure. 1084 98

Erythroderma can be caused by a variety of underlying dermatoses, infections, and systemic diseases. Many of the findings on history, physical examination, and laboratory evaluation are nondiagnostic. Distinctive clinical and laboratory features pointing to a specific disease may be evident, however. Conclusive clinicopathologic correlation may require multiple and repeated skin biopsies. The prognosis of erythroderma has improved with the advent of innovative dermatologic therapies (e.g., cyclosporine and synthetic retinoids) and advances in the management of systemic manifestations. Death from sepsis, cardiac failure, adult respiratory distress syndrome, and capillary leak syndrome continue to be rarely reported. A high index of suspicion for these complications must be maintained to facilitate early medical intervention.
Dermatol Clin 2000 Jul
PMID:Erythroderma. 1094 36

The epidermolysis bullosa-pyloric atresia-obstructive uropathy (EB-PA-OU) association is a rare, but well-described multisystem disease. While the prognosis at this time is still poor, an increasing number of patients are surviving to adolescence with aggressive care. It is important to understand this syndrome in order to anticipate medical complications and offer preventive strategies where possible. Prompt and expectant management of obstructive uropathy is crucial in these patients. Evidence of ureterovesicular obstruction may require bowel diversion, as excision of the obstructed ureterovesicular junction with reimplantation is often associated with a high risk of reobstruction. Many newborns succumb to sepsis or dehydration and electrolyte imbalance. Those infants who survive need close monitoring for the development of obstructive uropathy, failure to thrive, protein-losing enteropathy, respiratory compromise, and increased susceptibility to invasive infections. Once a clinical diagnosis is made, mutational analysis can confirm it and facilitate genetic counseling, as recurrence risks are 25% for this autosomal recessive condition. Mutational analysis enables direct genetic testing and accurate prenatal diagnosis. As more patients are studied, genotype/phenotype correlations may be possible.
Pediatr Dermatol
PMID:Epidermolysis bullosa, pyloric atresia, and obstructive uropathy: a report of two case reports with molecular correlation and clinical management. 1099 May 77

We report a patient with severe bilateral leg ulceration that was resistant to treatment. A biopsy confirmed the cause as calciphylaxis. Calciphylaxis refers to a syndrome of calcium deposition in the small and intermediate dermal vasculature which can lead to epidermal ischaemia, ulceration and necrosis. Most cases occur in those with chronic renal failure and secondary hyperparathyroidism. We describe the rare presentation of calciphylaxis in a patient with normal renal function and primary hyperparathyroidism who had many classical features. Unfortunately she developed gangrene, sepsis and died.
Clin Exp Dermatol 2000 Jul
PMID:Calciphylaxis in the absence of renal failure. 1101 92


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