Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0243026 (sepsis)
 52,417 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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

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

We report on a sixty-seven year old miner with pemphigus vulgaris characterised clinically by a three month history of relapsing oral lesions and blisters/erosions on the trunk, axillae and extremities, histologically by suprabasal cleavage due to acantholysis, immunologically by the epidermal intercellular net-like pattern due to deposits of IgG- and IgM-antibodies and complement C3 in the direct immunofluorescence as well as by serum antibodies to desmoglein 3 (130 KD) and plakoglobin (85 KD) by immunoblotting analysis. Silicosis has already been known for 6 years. In addition, antinuclear antibodies, anti-ssDNA-antibodies and anti-topoisomerase antibodies were found. Clinical improvement and clearing of skin symptoms could be achieved by systemic steroids in combination with cyclophosphamide. However, the patient died of sepsis deriving from recalcitrant pneumonia. Although the association of silicosis with various autoimmune diseases such as systemic sclerosis, systemic lupus erythematosus, rheumatoid arthritis and dermatomyositis has been reported many times, our patient is, to the best of our knowledge, the second case with features of the two diseases: pemphigus vulgaris and silicosis.
Eur J Dermatol 2000 Dec
PMID:Pemphigus vulgaris in association with silicosis. 1112 24

We report a fatal case of toxic epidermal necrolysis (TEN) resulting from a high dose of cytosine arabinoside (ARA-C). A 13-year-old girl with acute lymphocytic leukemia was treated according to the protocol of the BFM Group (BFM-95, HRG). On the fifth day after administration of a high dose of ARA-C (2 g/m2 intravenously every 12 hours), she developed bullous lesions on the hands and soles that disseminated, evolving to necrosis, sepsis, and death on the 22nd day. ARA-C is frequently associated with dermatologic toxicity, but this is only the second case of toxic epidermal necrolysis described in connection with this drug.
Pediatr Dermatol
PMID:Toxic epidermal necrolysis after the use of high-dose cytosine arabinoside. 1120 69

A 3-year-old girl receiving chemotherapy for acute lymphocytic leukaemia developed a rapidly expanding red annular plaque on her thigh, initially without signs of systemic toxicity or local pain. Subsequently she developed Pseudomonas aeruginosa sepsis and purpura at the leading edge of the plaque. Skin biopsy showed an extensive necrotizing vasculitis with numerous Gram-negative bacilli in the blood vessel walls. In immunocompromised individuals, skin biopsy and culture of cutaneous lesions for bacteria and fungi should be considered even in the absence of signs of systemic toxicity or multiple lesions.
Australas J Dermatol 2001 Feb
PMID:Pseudomonas aeruginosa infection mimicking erythema annulare centrifugum. 1123 25

A 50-year-old Japanese female with chronic renal failure who had been on continuous ambulatory peritoneal dialysis developed fulminant systemic cutaneous necrosis that began as painful livedo reticularis-like skin lesions on her thighs. Because of disseminated vascular calcification within the muscular layer of her lower limbs, we eventually diagnosed her with calciphylaxis. The skin necrosis progressed rapidly, and she died of sepsis and pneumonia on the 53rd hospital day. In addition to her long-lasting severe hyperparathyroidism and extremely elevated serum phosphorus and calcium levels, mechanical, frictional stimulation inflicted on the local skin and administration of corticosteroids were suspected to have precipitated the calciphylaxis. Our lack of awareness of this disease in its early stages resulted in our missing the chance to do a parathyroidectomy that might have changed the course. It is important to know the clinical features of this rare disease in order to make a diagnosis as early as possible.
J Dermatol 2001 Jan
PMID:Fulminant and relentless cutaneous necrosis with excruciating pain caused by calciphylaxis developing in a patient undergoing peritoneal dialysis. 1128 Apr 61


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