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)

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

Necrotizing cellulitis and fasciitis may be difficult to recognize. When skin necrosis is not obvious, the diagnosis must be suspected if there are signs of severe sepsis (accelerated heart or respiratory rates, oliguria, mental confusion.) and/or some of the following local symptoms or signs: severe spontaneous pain, indurated edema, bullae, cyanosis, skin pallor, absence of lymphangitis, skin hypoesthesia, crepitation, muscle weakness, foul smell of exudates. Many risk factors are suspected. A recent case-control study demonstrated that using ibuprofen increased the risk of cellulitis complicating chickenpox in children. Evidence is lower for other risk factors that are present with a high prevalence in most series: local lesion of skin or mucous membranes (acute or chronic disease, traumatism, surgery.), diabetes, arteriopathy, alcoholism, obesity, immunosuppression, NSAIDs. The risk of streptococcal necrotizing fasciitis is increased when in contact with patients infected by the same streptococcus.
Ann Dermatol Venereol 2001 Mar
PMID:[Necrotizing fasciitis. Clinical criteria and risk factors]. 1131 68

Necrotizing fasciitis are characterized by the necrosis of fascias, and their severe consequences in terms of morbidity and mortality. An early diagnosis, based on sometimes subtle cutaneous lesions (associated to a sepsis syndrome) allows to start resuscitation and decide on a probable surgery. 3 major forms can be distinguished: streptococcal fasciitis, due to beta-hemolytic streptococci, often following minor trauma, and increasingly associated to a streptococcal toxic shock syndrome (STTS); clostridial gangrene (often polymicrobial when developed on a open wound or after surgery); and synergistic gangrene due to a mixed aerobic-anaerobic flora. Other apparently "primitive" necrotizing fasciitis, caused by specific organisms, may occur in debilitated patients. The prognosis depends on age, comorbidity, and above all on the severity of the sepsis syndrome. Initial resuscitation involves controlling the hypotension and organ dysfunction associated with severe sepsis, and is usually dominated by a severe hypovolemia. Penicillin G remains the key antibiotic for streptococcal and clostridial fasciitis, with a broad spectrum including enterobacteriaceae, streptococci and enterococci, and anaerobes (including Bacteroides spp.) in other types or when the etiology is unknown. In patients presenting with STSS, a combination of clindamycin (or rifampin) to penicillin is recommended, because of their effect on exotoxin production; administration of non-specific immunoglobulins also appears to improve the outcome of patients affected. Hyperbaric oxygen therapy has not proved effective. Early surgical debridement largely influences the prognosis. The prevention of complications associated with long-term intensive care, including early nutritional support and prevention of a thromboembolic disease, is also important.
Ann Dermatol Venereol 2001 Mar
PMID:[The therapeutic approach to necrotizing fasciitis]. 1131 71

Calciphylaxis is a rare and life-threatening condition of progressive cutaneous necrosis secondary to small and medium-sized vessel calcification. It is seen almost exclusively in patients with end-stage renal disease and secondary hyperparathyroidism. We experienced a case of 67-year-old man with calciphylaxis that manifested with characteristic skin lesions, pathologic findings, and laboratory changes. His skin lesions began as painful erythematous patches and subsequently progressed to necrotic ulcers with eschars on the distal aspect of the extremities. Pathologically, calcification was found in small and medium-sized blood vessels in the deep dermis and subcutaneous tissue. His serum calcium was 9.5 mg/dL, phosphorus was 7.8 mg/dL, and nPTH was 99.9 pg/mL. The patient had been treated with surgical debridement and other supportive treatment. However, he eventually underwent an amputation below the right knee and died from sepsis.
J Dermatol 2001 May
PMID:Calciphylaxis in a patient with end-stage renal disease. 1143 66

A 15-day-old Yemeni boy presented with anonychia and granulomatous nail beds and white patches in the mouth. Biopsy specimens from the nail beds were nondiagnostic. Shortly thereafter the child developed multiple tense bullae, a hoarse voice, and poor appetite. Hematoxylin and eosin staining along with monoclonal antibody studies of a skin biopsy specimen revealed subepidermal bullae through the lamina lucida and a marked decrease in laminin 5. A diagnosis of junctional epidermolysis bullosa Herlitz variant was made. His course was complicated by multiple nonhealing wounds, oral pharyngeal involvement, sepsis, anemia, and poor nutrition, leading to his eventual death. This report emphasizes the unusual presentation of Herlitz junctional epidermolysis bullosa with anonychia as the initial finding and a relatively prolonged period before cutaneous blister formation, resulting in delay of diagnosis.
Pediatr Dermatol
PMID:Herlitz junctional epidermolysis bullosa presenting at birth with anonychia: a case report and review of H-JEB. 1143 2

Epidermolysis bullosa dystropicans of the Hallopeau-Siemens type (HS-EBD) is an autosomal-recessive blistering disease. Skin fragility due to mutations in structural proteins is responsible for further development of chronic and painful wounds, skin infections and sepsis. There is no causative treatment available. We present a case report with HS-EBD and longstanding painful wounds treated with autologous keratinocytes on an esterified hyaluronic acid membrane. Two out of three wounds treated showed a complete take of the graft. They improved markedly with a stable result over 12 months until now. Even autologous keratinocyte grafting may have a beneficial effect on chronic wounds in HS-EBD despite the fact that the genetic defects are unchanged.
J Dermatol 2001 Apr
PMID:Recessive epidermolysis bullosa dystrophicans (Hallopeau-Siemens)--improvement of wound healing by autologous epidermal grafts on an esterified hyaluronic acid membrane. 1144 73

The disease of Epstein-Barr virus (EBV) -associated recurrent necrotic papulovesicles is a distinct clinicopathologic entity different from classic hydroa vacciniforme (HV). A few patients have been reported as atypical HV with systemic involvement, development of lymphoma, and poor prognosis. We describe a patient with recurrent necrotic papulovesicles and multiple varioliform scars in both sun-exposed and covered areas. In contrast to cases of previously reported atypical HV, our patient suffered from repeated bacterial infections on various sites ending in sepsis and death, but without malignant transformation. EBV was detected in the lymphoid cells from the skin lesions by anti-latent membrane protein (LMP) antibody and in situ hybridization. We suggest that the repeated bacterial infections in this case raise the possibility of an association of EBV infection with increased susceptibility to bacterial infections.
J Dermatol 2001 Aug
PMID:Epstein-Barr virus-associated recurrent necrotic papulovesicles with repeated bacterial infections ending in sepsis and death: consideration of the relationship between Epstein-Barr virus infection and immune defect. 1156 Jan 62


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