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Query: UMLS:C0036690 (sepsis)
 59,461 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report the case of a patient with pemphigus who presented Nocardia asteroides septicemia. The infection was controlled with an original association of trimethoprim-sulfamethoxazole and amikacin.
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PMID:Nocardia asteroides septicemia in a pemphigus patient. Successful treatment with trimethoprim-sulfamethoxazole and amikacin association. 207 62

To determine the potential steroid sparing effect of plasma exchange in pemphigus we enrolled 40 patients in a multicenter randomized study. Eighteen patients were treated by prednisolone alone, 22 by prednisolone plus ten large-volume plasma exchanges over four weeks. All patients received oral prednisolone in the same initial dosage (0.5 mg/kg/d), which was increased weekly if needed. The number of cases controlled at each therapeutic step did not differ between the two groups. In eight cases, four in each group, the disease was not controlled by the highest therapeutic step of the protocol, with four deaths from sepsis in the plasma exchange group. The controlled cases needed similar cumulative prednisolone doses (5237 +/- 5512 mg in the plasma exchange group vs 4246 +/- 1601 mg in the control group). The evolution of serum pemphigus antibody was not different in the two groups. These findings suggest that plasma exchange in association with low steroid doses alone are not effective in the treatment of pemphigus and may even promote sepsis.
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PMID:Controlled study of plasma exchange in pemphigus. 317 48

A 42-year-old woman with extensive pemphigus vulgaris failed to respond to high-dosage systemic steroids, immunosuppressive drugs and plasmapheresis. The treatment resulted in severe depression of the immune system, without marked effect on the disease activity. Eleven weeks after onset of disease she died of pneumonia and sepsis.
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PMID:[Pemphigus vulgaris--malignant course]. 353 47

Eighteen autopsies were performed in patients with pemphigus that died during treatment between 1961 and 1981. Eight of these patients had endemic pemphigus foliaceus and ten had pemphigus vulgaris. The pemphigus vulgaris patients were receiving only corticosteroids while some of those with pemphigus vulgaris also received immunosuppressors. Side effects from the medication were a major contributing factor in the death of 7 of the 8 pemphigus foliaceus patients and in 8 of the 10 cases of pemphigus vulgaris. The causa mortis to which the medication contributed to was strongyloidiasis in 4 patients, disseminated septicemia in 3 patients, bronchial pneumonia in 3 patients, miliary tuberculosis in 2 patients, massive gastrointestinal hemorrhage, pulmonary infarct and balantidiasis with strongyloidiasis in 1 patient each. The authors stress the need for careful investigation of strongyloidiasis and eradication by treatment not only when starting but also during therapy with large doses of corticosteroids and cytotoxic agents.
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PMID:[Post-mortem evaluation in endemic pemphigus foliaceus and pemphigus vulgaris]. 637 24

A 62 year old female was treated for several years with DDS because of Duhring-disease like symptoms. She developed erythro-melanoderma, generalized lymphadenitis and pachyderma. Histologically there were eosinophilic spongiosis and intraepidermal vesiculation, blood eosinophilia up to 42%, and intercurrent streptococcal septicemia, immunohistologically in vivo bound pemphigus antibody, but no papillary or linear IgA. Indirect immunofluorescence was negative. Paraproteinemia was excluded.
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PMID:[Erythromelanoderma in pemphigus herpetiformis associated with eosinophilic spongiosis]. 644 27

Pemphigus is frequently a fatal skin disease. The cause of death and the events leading to it were investigated by examining patient records and autopsy data of thirteen patients who died as a result of this disease at the UCLA Hospital between 1965 and 1980. The diagnosis was confirmed histologically and/or by immunofluorescent studies of the skin and serum. Infection was the most frequent cause of death, and septicemia was found in nine of thirteen cases. The most commonly found organism was Staphylococcus aureus. The skin was usually the source of infection. Nine patients had pneumonia on chest x-ray and autopsy examination. Most patients had low levels of serum proteins and serum albumin. Since the patients were on long-term high doses of corticosteroids, the signs and symptoms of inflammation were often masked. This study demonstrated that for this reason, long-term corticosteroid therapy is one of the significant factors contributing to the death of these patients. A cautious and judicious use of steroids is suggested.
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PMID:Death in pemphigus. 713 Apr 83

The records of 10 patients who died with pemphigus have been examined for factors affecting and contributing to death. Early initiation of therapy, the age at onset of the disease, and the mode of administration of corticosteroids, conventional or in pulse form, did not affect the survival. The cutaneous involvement was extensive in all 10 patients; it ranged between 30-80%. Septicemia was the commonest event preceding death; in 4 cases, it was due to Staphylococcus aureus.
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PMID:Factors responsible for death in patients with pemphigus. 796 69

We report on a 45-year-old patient, who first developed pemphigus vegetans of the Neumann type 9 years ago and who presented with typical manifestations of the disease 6 years after the first exacerbation. On systemic therapy with corticosteroids progression of the disease ceased. Additional application of azathioprine, cyclophosphamide or gold thiomalate, however, did not make it possible to reduce the high corticosteroid dosage without exacerbation of the disease. The long-term corticosteroid therapy led to sepsis, thrombosis of the leg and pelvic veins and osteoporosis with crush fractures of the vertebrae; corticosteroid therapy was therefore discontinued and replaced by cyclophosphamide bolus therapy associated with gold thiomalate. The subsequent exacerbation of the pemphigus was treated with methotrexate and dapsone. The skin lesions progressed further, however, and the patient died of sepsis 39 weeks after admission to the clinic.
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PMID:[Neumann type pemphigus vegetans with fatal outcome]. 844 2

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.
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PMID:Pemphigus vulgaris in association with silicosis. 1112 24

Blistering dermatitises are characterized by the presence of blisters that begin owing to acantholysis (intraepidermic blister) such as pemphigus vulgaris (PV) or owing to dermoepidermic detachment (subepidermic blister) such as bullous pemphigoid (BP). Both diseases are autoimmune pathologies characterized by the presence of autoantibodies against specific adhesion molecules of the skin and mucous membranes. PV, in which oral lesions are always present, has a progressive course that, if the disease is not treated, nearly always brings to death from sepsis within a few years. In BP, oral lesions are rare and the disease, that is most frequent in older individuals, has a chronic course with spontaneous remissions. Systemic corticosteroids and immunosuppressants are the mainstay of treatment of these two diseases. Although this therapy had reduced the mortality of the two pathologies it is associated with serious side effects. To reduce the corticosteroids dose and to improve the symptomatology in resistant therapy cases, we treated five patients with several procedures of plasma exchange. Four patients were affected by BP and one by PV. Their disease severity at onset of plasmapheresis ranged from mild to severe. One of 5 patients suffered a plasmapheresis side effect. All patients responded with complete remission of symptomatology and had a prednisone dosage reduction until 70%. Plasmapheresis is an effective treatment for PV and BP patients who have been unresponsive to conventional therapy, for those for whom conventional drugs are contraindicated, for those who show severe clinical manifestations and for those who need high doses of corticosteroids and immunosuppressants to keep the disease under control.
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PMID:Plasmapheresis therapy in pemphigus vulgaris and bullous pemphigoid. 1262 Feb 64


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