1/45. in vitro released interferon-gamma in the diagnosis of drug-induced anaphylaxis.A 17-year-old Japanese male was referred with acute urticaria and anaphylaxis after the administration of PL (salicylamide, acetaminophen, anhydrous caffeine and promethazine methylene disalicylate) and Bufferin (aspirin and dialminate) for headache and a high grade fever. The results of prick test, patch test and drug-induced lymphocyte stimulation test with PL and Bufferin were all negative. The patient's peripheral blood mononuclear cells (PBMC) were cultured with or without PL for 72 hours, and the activity of interferon-gamma (IFN-gamma) in the culture supernatant was measured with EIA. A significantly high level of IFN-gamma was detected in PBMC from the patient, but very little in those from healthy control subjects with a history of exposure to PL. This finding may indicate the presence of drug-specific IFN-gamma producing T cells in patients with an anaphylactic shock reaction to medication. Assays that measure the drug-induced IFN-gamma production may thus be a useful diagnostic tool not only for identifying delayed-type hypersensitivity (DTH) to drugs, but also for predicting anaphylactic shock reaction to drugs.- - - - - - - - - - ranking = 1keywords = fever (Clic here for more details about this article) |
2/45. urticaria as a presenting manifestation of adult-onset Still's disease.adult-onset Still's disease (AOSD) is a rare disorder of unknown aetiology, characterised by high spiking fever, an evanescent, erythematous, maculopapular rash, arthralgia or arthritis, lymphadenopathy, hepatosplenomegaly, sore throat and serositis. It is associated with marked leukocytosis, high erythrocyte sedimentation rate, increased level of serum ferritin and negative rheumatoid factor and antinuclear antibody tests. Here we report a patient in whom an urticaria-like rash was an uncommon presenting clinical feature of AOSD. To our knowledge, this association has only been reported once before.- - - - - - - - - - ranking = 1keywords = fever (Clic here for more details about this article) |
3/45. The schnitzler syndrome. Four new cases and review of the literature.The schnitzler syndrome is characterized by a chronic urticarial eruption with a monoclonal IgM gammopathy. The other signs of the syndrome include intermittent elevated fever, joint and/or bone pain with radiologic evidence of osteosclerosis, palpable lymph nodes, enlarged liver and/or spleen, elevated erythrocyte sedimentation rate, and leukocytosis. The mean delay to diagnosis is more than 5 years, and this syndrome is of concern to internists and many medical specialists. patients with this syndrome are often initially considered to have lymphoma or adult-onset Still disease, which are the main differential diagnoses. However, hypocomplementic urticarial vasculitis, systemic lupus erythematosus, cryoglobulinemia, acquired C1 inhibitor deficiency, hyper IgD syndrome, chronic infantile neurologic cutaneous and articular (CINCA) syndrome, and Muckle-Wells syndrome should also be excluded, because diagnosis relies on a combination of clinical and biologic signs and there is no specific marker of the disease. The disease pursues a chronic course, and no remissions have yet been reported. Disabling skin rash, fever, and musculoskeletal involvement are the most frequent complications. Severe anemia of chronic disease is another serious complication. The most harmful complication, however, is evolution to an authentic lymphoplasmacytic malignancy, which occurs in at least 15% of patients. This hematologic transformation can occur more than 20 years after the first signs of the disease, thus patients deserve long-term follow-up. Treatment is symptomatic and unsatisfactory. The skin rash is unresponsive to treatment, and nonsteroidal antiinflammatory drugs, antihistamines, dapsone, colchicine, and psoralens and ultraviolet A (PUVA) therapy give inconstant results. Fever, arthralgia, and bone pain often respond to nonsteroidal antiinflammatory drugs. In some patients, these symptoms and/or the presence of severe inflammatory anemia require steroids and/or immunosuppressive treatment, which ameliorate inflammatory symptoms but do not change the course of the skin rash.- - - - - - - - - - ranking = 2keywords = fever (Clic here for more details about this article) |
4/45. association between terbinafine and arthralgia, fever and urticaria: symptoms or syndrome?PURPOSE: The antifungal agent terbinafine has been approved for marketing in The netherlands since 1992. Adverse drug reactions (ADRs) may occur in about 10% of the patients, the majority gastrointestinal disorders and skin reactions. Since the introduction of terbinafine, the netherlands pharmacovigilance Foundation Lareb received eight reports of arthralgia during the use of this drug. In four reports the additional presence of skin reactions was mentioned, two of these reports concerned urticaria. Two patients who reported arthralgia also had a fever. These reports were described in more detail, and analysed statistically in order to determine whether symptoms are interrelated. methods: All reports with known gender and a reporting date between 1 March 1992 and 1 January 1999, concerning patients older than 10 years, were included. The extent to which the symptoms urticaria, fever and arthralgia were interrelated was examined by logistic regression modelling. RESULTS: Case series as well as the results of the statistical analysis show a clustering of symptoms among reports of patients using terbinafine. Both urticaria and arthralgia were statistically significantly associated with reports on terbinafine compared to all other reports in the database. CONCLUSION: The findings might point towards a clustering of these symptoms in patients using terbinafine. Possibly these symptoms have a shared aetiology, presumably an immunological reaction.- - - - - - - - - - ranking = 6keywords = fever (Clic here for more details about this article) |
5/45. Malignant evolution of Schnitzler's syndrome--chronic urticaria and IgM monoclonal gammopathy: report of a new case and review of the literature.Schnitzler's syndrome, initially described in 1974 is an uncommon condition defined by chronic urticaria and monoclonal IgM gammopathy. Additional features include fever of unknown origin, elevated ESR, bone pain and frequently a benign clinical course. We conducted a literature search of medline, EMBASE and Cancerlit and found 56 cases of Schnitzler's syndrome reported to date. The absence of lymphoproliferative disease in this condition is typical, but nine patients have progressed to develop lymphoplasmacytic neoplasias, particularly Waldenstrom's macroglobulinemia (WM). Malignant evolution of Schnitzler's syndrome is a rare complication, but emphasizes the importance of long term follow-up and the need for these patients to undergo periodic assessment of the bone marrow and lymph nodes. Treatment of this condition is difficult, with varying response to corticosteroids and largely unsuccessful results with standard chemotherapy used for WM. We describe a case of Schnitzler's syndrome in a 50-year old man with lymphocytic aggregates in the bone marrow after 9 years of chronic urticaria, fever, arthralgias and bone pain. We review the clinical features and treatment, with emphasis on the hematologic aspects of this unusual condition.- - - - - - - - - - ranking = 2keywords = fever (Clic here for more details about this article) |
6/45. Recurrent urticaria as a rare manifestation of familial mediterranean fever.familial mediterranean fever (FMF) is a genetic disorder characterized by acute episodes of fever with some combination of severe abdominal pain, pleurisy, arthritis, and skin rash. The case of a patient with recurrent urticaria referred for study of drug allergy is presented. After allergy had been ruled out, the urticaria was attributed to previously undiagnosed symptoms of an underlying systemic disease: FME. urticaria is the least frequent cutaneous manifestation of this disease, and genetic analysis was required to confirm the diagnosis.- - - - - - - - - - ranking = 6keywords = fever (Clic here for more details about this article) |
7/45. A large kindred with familial cold autoinflammatory syndrome.BACKGROUND: Familial cold autoinflammatory syndrome (FCAS), formerly known as familial cold urticaria, is a rare condition characterized by fever, rash, and arthralgias elicited by exposure to cold. Recently, mutations responsible for FCAS were identified in a novel gene (CIAS1), making it possible to confirm the diagnosis in most patients. OBJECTIVE: We present a summary of clinical data from a large family with FCAS to further define the characteristics of the disorder and to validate previously proposed clinical criteria. methods: A total of 73 participants were evaluated by interview and questionnaire, including 36 affected individuals. Responses from the questionnaire were analyzed and comparisons of proportions were made using the Z test. dna was isolated and genotyping was performed on all subjects. Affected haplotypes (genotype patterns) were identified and used to confirm the diagnosis. Sequencing of the CIAS1 gene was performed in selected patients to confirm the mutation. RESULTS: The prevalence of rash, fever/chills, joint complaints, nausea, headache, and thirst were not significantly different from previously reported proportions. There was statistically significant differences in conjunctivitis, sweating, and drowsiness with alpha = 0.01. The mean temperature required to produce symptoms was 22 degrees C, and the average earliest onset of symptoms after exposure was 1.5 hours. CONCLUSIONS: Applying the proposed clinical criteria, 41% of affected subjects met all six criteria, 90% met five criteria, and 100% met four criteria for FCAS. None of the unaffected subjects met more than two criteria. Using a threshold of 4 of 6 clinical criteria, the data support the diagnostic validity of the proposed clinical criteria.- - - - - - - - - - ranking = 3342.0273803792keywords = autoinflammatory, fever (Clic here for more details about this article) |
8/45. CIAS1 mutation in a patient with overlap between Muckle-Wells and chronic infantile neurological cutaneous and articular syndromes.The Muckle-Wells syndrome is a rare autosomal dominant disorder belonging to the group of hereditary fever syndromes. The chronic infantile neurological cutaneous and articular (CINCA) syndrome is a systemic inflammatory disorder of unknown etiology with neonatal onset. They are considered as two different entities. We report the case of a 36-year-old man suffering since birth from a nonpruritic generalized urticaria, with inflammatory flares, joint manifestations and progressive deafness requiring a bilateral hearing aid. An initial diagnosis of Muckle-Wells syndrome was made. However, the patient had an unusual clinical presentation with slightly dysmorphic facial appearance, clubbing of the fingers, mild mental retardation and papilledema. After a genetic advice, a diagnosis of CINCA syndrome was made. Search for mutations in the CIAS1 gene revealed a new mutation in a heterozygous state. This case report really raises the question of a link between these two inflammatory diseases. Further studies are needed to confirm the involvement of mutations of the CIAS1 gene in CINCA syndrome.- - - - - - - - - - ranking = 1keywords = fever (Clic here for more details about this article) |
9/45. A near fatal case of the dapsone hypersensitivity syndrome in a patient with urticarial vasculitis.dapsone (4,4'-diaminodiphenyl sulphone) is used for a variety of dermatological conditions including immunobullous diseases and urticarial vasculitis. Side-effects are common and include lethargy, headaches, methaemoglobinaemia and haemolysis. Severe adverse effects are rare but the dapsone hypersensitivity syndrome is well recognized. Symptoms include fever, haemolytic anaemia, lymphocytosis and hepatitis. We report a near fatal case of the dapsone hypersensitivity syndrome in a patient with urticarial vasculitis. This diagnosis should be remembered in any patient who becomes unwell whilst taking dapsone.- - - - - - - - - - ranking = 1keywords = fever (Clic here for more details about this article) |
10/45. hepatitis b-assocciated adult-onset Still's disease presenting with neutrophilic urticaria.adult-onset Still's disease (AOSD) is an uncommon systemic inflammatory disorder that is characterized by quotidian fever, articular manifestations, neutrophilic leukocytosis, and maculopapular rash. The aetiology of the disease is unknown, however, an infectious cause has been suggested. Here we describe a patient in whom neutrophilic urticaria was the cutaneous manifestation of AOSD. In addition, the patient suffered from chronic hepatitis b infection that may be a potential trigger factor of AOSD. In patients with AOSD, serological investigations for detection of infection should include hepatitis serology. Further, we suggest that urticarial lesions may be a more common cutaneous manifestation of AOSD than has been recognized previously. Thus it is important to include AOSD in the differential diagnosis of urticaria.- - - - - - - - - - ranking = 1keywords = fever (Clic here for more details about this article) |
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