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)

Pseudomonas aeruginosa bacteremia or sepsis often occurs in hospitals, affecting mainly children with underlying disease. Ecthyma gangrenosum is classically considered a pathognomonic sign of sepsis by P. aeruginosa. The harlequin baby, a severe variant of ichthyosis, occurs rarely, and these infants are at high risk of cutaneous infections and sepsis. We herein report a harlequin baby who developed ecthyma gangrenosum.
Pediatr Dermatol
PMID:Harlequin baby with ecthyma gangrenosum. 1465 76

Pemphigus, a rare, chronic blistering disease of the skin and mucous membranes with severe morbidity and occasional mortality, is the most common autoimmune bullous disease in Korea. The purpose of this study was to evaluate the clinical features and propose a treatment strategy for patients with pemphigus. A retrospective analysis was conducted of 51 pemphigus patients seen between 1993 and 2001. Pemphigus vulgaris (PV) was the most common type with 32 cases, followed by 19 cases of pemphigus foliaceus (PF). The male to female ratio was 1:1.3, with females predominating, particularly among PV patients (PV, 1:1.5; PF, 1:1.1). The average ages at onset of PV and PF were 44.3 and 51.0 years old, respectively. Mucosal involvement was noted in 27 cases (84.4%) of PV but in only 3 cases (15.8%) of PF. Most patients initially received relatively low to intermediate doses (0.3-1.0 mg/kg/day) of prednisolone, and 23 (71.9%) PV patients and 10 (52.6%) PF patients also received immunosuppressive agents. Oral prednisolone and azathioprine (100 mg/day) formed the mainstay of treatment for our patients (47.1%). At the time of writing, 25.5% (13/51) of patients are in complete remission, and 72.5% (37/51) are undergoing maintenance therapy. One patient died due to sepsis during the treatment. For the treatment of pemphigus, a course of the lowest possible corticosteroid dosage in combination with immunosuppressive agents appears to be effective and less toxic than a high corticosteroid dosage.
J Dermatol 2003 Nov
PMID:Pemphigus in Korea: clinical manifestations and treatment protocol. 1468 34

We present a case of a 78-year-old man suffering from a chronic psoriasiform eruption, with rapid deterioration over the previous 8 weeks. Langerhans' cell histiocytosis with skin and bone involvement was diagnosed, and there was evidence of liver and lung dysfunction. The patient was treated with prednisolone and etoposide, and initially experienced a partial improvement. Three weeks later, haemophagocytic lymphohistiocytosis and subsequently a large pulmonary abscess with sepsis attributed to opportunistic gram-negative enterobacteriaceae Serratia marcescens developed, and the patient died. The present case of Langerhans' cell histiocytosis is of particular interest because of the previously unreported development of haemophagocytic lymphohistiocytosis in the elderly population.
J Eur Acad Dermatol Venereol 2003 Nov
PMID:Langerhans' cell histiocytosis and haemophagocytic lymphohistiocytosis in an elderly patient. 1476 Nov 43

Ecthyma gangrenosum is a cutaneous infection associated most commonly with pseudomonal sepsis in the patient who is immunocompromised. We describe an 8-month-old girl with acute myelocytic leukemia who developed perineal ecthyma gangrenosum caused by Citrobacter freundii, a gram-negative pathogen that has been rarely associated with cutaneous disease. We also review the literature to categorize the range of pseudomonal and nonpseudomonal pathogens associated with ecthyma gangrenosum.
J Am Acad Dermatol 2004 May
PMID:Nonpseudomonal ecthyma gangrenosum. 1509 44

Circulating human neutrophils from patients with severe inflammatory disorders such as erysipelas and sepsis are specifically desensitized to complement factor C5a stimulation but not to stimulation with other stimuli like N-formyl-methionyl-leucyl-phenylalanine (FMLP), interleukin-8 (IL-8), leukotriene B4 (LTB4), or platelet-activating factor (PAF, 1-O-alkyl-2-acetyl-sn-glycero-3-phosphocholine). In this study, we raised the question whether factors released from polymorphonuclear leukocytes (PMNs) can specifically down-regulate C5a-dependent neutrophil functions. When neutrophils were preincubated with either neutrophil lysates or neutrophil degranulation supernatants, a complete inhibition of C5a-stimulated beta-glucuronidase release and chemotaxis could be observed, whereas FMLP-, IL-8-, LTB4- or PAF-dependent functions were not affected. Serine protease inhibitors like phenylmethylsulfonyl fluoride, antileukoprotease, or elafin abolished this effect. High-performance liquid chromatography of neutrophil degranulation supernatants revealed pronounced inhibition of C5a-dependent neutrophil functions in fractions exerting elastase or cathepsin G activity, but not in fractions exerting proteinase 3 activity. Using purified human leukocyte elastase (HLE), C5a responses like intracellular calcium influx, beta-glucuronidase release, and chemotaxis were also specifically inhibited. Our experiments show that the release of HLE or cathepsin G from neutrophils specifically down-regulates the responsiveness of neutrophils to C5a. Elastase and cathepsin G may therefore play an important role in the down-regulation of acute inflammation.
Exp Dermatol 2004 May
PMID:Human leukocyte elastase and cathepsin G are specific inhibitors of C5a-dependent neutrophil enzyme release and chemotaxis. 1514 22

Since 1991 infrequent reports have described a distinctive triad of nodulocystic acne, striking follicular spines and an eruption resembling pityriasis rubra pilaris (PRP) in HIV-positive patients. It has been suggested that this may represent a subtype of PRP, or alternatively that it should be viewed as a unique HIV-associated follicular occlusion triad. Clinical manifestations may be severe, and in several cases have been ultimately fatal, with death occurring due to complications of cutaneous sepsis. We describe a case demonstrating severe conglobate acne, follicular keratotic spines and histologically confirmed PRP in association with HIV infection. Clinical features and treatment modalities of previously reported cases are reviewed. Despite refusing all topical and systemic treatment our patient showed spontaneous remission of skin signs after 2 years.
Clin Exp Dermatol 2004 Sep
PMID:Spontaneous clinical improvement in HIV-associated follicular syndrome. 1534 29

We report a woman with recessive dystrophic epidermolysis bullosa (RDEB) in whom there was prolonged sepsis and death at age 22 years. Autopsy revealed multiple epidermolytic skin lesions with chronic ulceration, mesangioproliferative glomerulonephritis and multifocal necrotizing leucoencephalopathy (MNL) of the pons. The latter two conditions may have been mediated by sepsis-associated cytokines. Although mesangioproliferative glomerulonephritis has previously been described in association with RDEB, to our knowledge this is the first report of MNL in a patient with RDEB.
Br J Dermatol 2004 Dec
PMID:Recessive dystrophic epidermolysis bullosa associated with mesangioproliferative glomerulonephritis and multifocal necrotizing leucoencephalopathy of the pons. 1560 25

Toxic epidermal necrolysis (TEN) is very rare in the newborn period. So far, three cases of TEN in newborns have been reported worldwide. We report a premature infant of 27 weeks' gestational age with TEN at 4 weeks of age. Sepsis treated by an antibiotic combination regimen preceding the TEN was a common feature of all four cases. In our patient, coagulase-negative staphylococci could be identified by blood culture, whereas the previously reported patients suffered from Klebsiella pneumoniae sepsis or Escherichia coli sepsis. Possibly, the uniform association with septic infection in the cases of TEN in the neonatal period might hint at a causal association, thus differentiating it from TEN in older children or adults.
Br J Dermatol 2005 Jan
PMID:Toxic epidermal necrolysis in a premature infant of 27 weeks' gestational age. 1565 16

Epidermolytic hyperkeratosis is an unusual type of ichthyosis. This inherited keratinization disorder is characterized clinically by erythema, blistering, and peeling shortly after birth. It may resolve and be replaced with thick scaling. It can lead to life-threatening complications, such as sepsis. Histologically, there is a hyperkeratosis and vacuolar degeneration. Genetically, this is an autosomal dominant disease with complete penetrance; however, 50% are spontaneous mutations. The clinical phenotype is a result of alterations in the gene(s) for keratin 1 and/or 10. We review this disorder and its therapy, which is mainly symptomatic with emollients and retinoids.
Int J Dermatol 2005 Jan
PMID:Epidermolytic hyperkeratosis: a keratin 1 or 10 mutational event. 1566 49

The tropical diabetic hand syndrome (TDHS) is a complication affecting patients with diabetes mellitus in the tropics. The syndrome encompasses a localized cellulitis with variable swelling and ulceration of the hands, to progressive, fulminant hand sepsis, and gangrene affecting the entire limb. TDHS is less well recognized than foot infections and not generally classified as a specific diabetes complication. Hand infection was first described in Nigeria in 1984. Since then, the majority of cases have been reported in the African continent and more recently in India. There is often a history of antecedent minor hand trauma (e.g. scratches or insect bites). Presentation to hospital is often delayed due to the patients' unawareness of the potential risks, lack of concern because the initiating trauma might have been trivial, or decision to seek initial help from traditional healers. The first analytic study was done in Dar es Salaam, Tanzania, to characterize the epidemiology, clinical characteristics and risk factors of TDHS. Independent risk factors for TDHS include poorly controlled diabetes, neuropathy, insulin treatment or malnutrition. Clinicians should be aware of these complications and be prepared to immediately admit TDHS patients to hospital for aggressive surgical intervention (i.e. debridement, pus drainage or amputation) and high-dose, intravenous, broad-spectrum antibacterial therapy that includes anti-anaerobic activity. Without prompt, aggressive treatment TDHS can lead to permanent disability, limb amputation (13% of TDHS patients require major upper limb amputation), or death. Prevention strategies include patient and staff education that focuses on proper hand care, nutrition, and the importance of seeking medical attention immediately following hand trauma regardless of the severity of the injury, or at the earliest onset of hand-related symptoms, such as redness or swelling. Prevention of permanent disability and death due to TDHS will require improved management of glycemic levels in resource-limited countries, and surgical intervention during less severe stages of the condition.
Am J Clin Dermatol 2005
PMID:Tropical diabetic hand syndrome. Epidemiology, pathogenesis, and management. 1567 87


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