1/56. Chronic urticaria as a presenting sign of hairy cell leukemia.Chronic urticaria is a common clinical disorder that is idiopathic in over 75% of cases. Less commonly, urticaria may be the presenting manifestation of an allergic or infectious disease, endocrinopathy, inherited syndrome, or autoimmune disorder. Rarely, urticaria may be a sign of underlying malignancy, including leukemia. C.C. is a 48-year-old white female who was referred for evaluation of recurrent urticaria for 3 years. The pruritic, erythematous wheals were pinpoint, and appeared to be precipitated by heat, stress, and effort. Prick tests were negative except to D. pteronyssinus. CBCs over the past 5 years revealed WBCs of 2,300-5,000 cells/mm3. Skin biopsy revealed interstitial edema with infiltration of eosinophils and mast cells consistent with urticaria. The impression was probable cholinergic urticaria, for which hydroxyzine was prescribed with fair symptomatic control. One year later, she presented with bright red blood per rectum. Repeat physical examination revealed lymphadenopathy and splenomegaly. Subsequent laboratory studies showed pancytopenia. endoscopy was normal except for small, nonbleeding hemorrhoids. bone marrow biopsy revealed histologic evidence of hair, cell leukemia that was treated with 2-chlorodeoxyadenosine. Upon initiation of chemotherapy her pruritus and urticaria subsided. Recent CBC revealed Hgb 9.2 g/dL, platelets 290,000 cells/mm3, and WBC 4,100 cells/mm3. Peripheral blood smear showed no hairy cells.- - - - - - - - - - ranking = 1keywords = idiopathic (Clic here for more details about this article) |
2/56. Persistent solar urticaria. A case report.Solar urticaria is an uncommon dermatological disease characterized by wheals developing within a few minutes after sun exposure and lasting a few hours. We describe a man in whom wheals developed on his trunk and arms more than 30 min after sun exposure and lasted more than 24 h. High doses of UVA reproduced lesions with histological features typical of urticaria. After 7 years, urticaria began to develop even in winter and without sun exposure. Our patient is unusual in that his wheals were delayed in onset and longlasting. The later association of idiopathic urticaria is an additional unusual feature.- - - - - - - - - - ranking = 1keywords = idiopathic (Clic here for more details about this article) |
3/56. Acquired lipoprotein lipase deficiency associated with chronic urticaria. A new etiology for type I hyperlipoproteinemia.Type I hyperlipoproteinemia (type I HLP) is a rare disorder of lipid metabolism characterized by fasting chylomicronemia and reduced postheparin plasma lipoprotein lipase (LPL) activity. Most cases of type I HLP are due to genetic defects in the LPL gene or in its activator, the apolipoprotein CII gene. Several cases of acquired type I HLP have also been described in the course of autoimmune diseases due to the presence of circulating inhibitors of LPL. Here we report a case of type I HLP due to a transient defect of LPL activity during puberty associated with chronic idiopathic urticaria (CIU). The absence of any circulating LPL inhibitor in plasma during the disease was demonstrated. The LPL genotype showed that the patient was heterozygous for the D9N variant. This mutation, previously described, can explain only minor defects in the LPL activity. The presence of HLP just after the onset of CIU, and the elevation of the LPL activity with remission of the HLP when the patient recovered from CIU, indicate that type I HLP was caused by CIU. In summary, we report a new etiology for type I HLP - a transient decrease in LPL activity associated with CIU and with absence of circulating inhibitors. This is the first description of this association, which suggests a new mechanism for type I HLP.- - - - - - - - - - ranking = 1682.0476351922keywords = chronic idiopathic, idiopathic (Clic here for more details about this article) |
4/56. Rapid oral challenge-desensitization for patients with aspirin-related urticaria-angioedema.BACKGROUND: Acetylsalicylic acid (ASA), commonly known as aspirin, is indicated in the treatment of coronary artery disease (CAD). Many patients are denied treatment with ASA because of a history of ASA or nonsteroidal anti-inflammatory drug (NSAID)-induced urticaria or angioedema. OBJECTIVE: We sought to develop a safe and practical protocol to allow the administration of ASA to patients with a history of ASA- or NSAID-induced urticaria-angioedema. methods: Eleven subjects with a history of ASA- or NSAID-induced urticaria-angioedema were challenged-desensitized by oral protocols based on rapidly escalating doses of ASA. Most had CAD, one had a history of pulmonary embolism, and one had refractory chronic sinusitis and asthma. Starting doses ranged from 0.1 to 10 mg and were administered at intervals of 10 to 30 minutes. Dosing was individualized for each patient but followed this general sequence (in milligrams): 0.1, 0.3, 1, 3, 10, 20, 40, 81, 162, 325. RESULTS: Nine patients tolerated the procedure without adverse effects and continued taking ASA for periods ranging from 1 to 24 months, without development of urticaria or angioedema. A patient who had a history of chronic idiopathic urticaria in addition to aspirin-induced urticaria had chest tightness during the protocol. Another patient who had continuing urticaria and angioedema associated with antithyroid antibodies developed angioedema several hours after completing the protocol. CONCLUSION: In patients with historical ASA- or NSAID-induced urticaria-angioedema reactions but who did not have urticaria and angioedema independent of ASA/NSAID, rapid oral challenge-desensitization to ASA was performed safely and permitted patients with CAD and other diseases to receive treatment with ASA.- - - - - - - - - - ranking = 1682.0476351922keywords = chronic idiopathic, idiopathic (Clic here for more details about this article) |
5/56. Idiopathic anaphylaxis: variants as diagnostic and therapeutic problems.Idiopathic anaphylaxis presents a problem requiring rapid diagnosis and initiation of therapy. Some cases are complex and difficult to assess. We present four cases of unusual complexity to illustrate diagnostic and therapeutic problems. Two cases were found not to be idiopathic anaphylaxis, one being undifferentiated somatoform idiopathic anaphylaxis and the other very severe urticaria. Various conditions can be or mimic idiopathic anaphylaxis, and patience and observation can result in reasonable outcomes.- - - - - - - - - - ranking = 3keywords = idiopathic (Clic here for more details about this article) |
6/56. Etanercept and urticaria in patients with juvenile idiopathic arthritis.Etanercept, a tumor necrosis factor receptor p75 Fc fusion protein (TNFR:Fc; Enbrel), has preliminarily been shown to be effective in the management of methotrexate-resistant polyarticular juvenile idiopathic arthritis (JIA). Reported side-effects have been minor, for example injection site reactions and upper respiratory tract infections, not necessitating discontinuation of the medication (1, 2). We report on 2 patients who developed an urticaria-like rash with prurigo appearing bilaterally on the extensor surfaces of the elbows subsequent to etanercept injections.- - - - - - - - - - ranking = 5keywords = idiopathic (Clic here for more details about this article) |
7/56. Chronic urticaria in children: expanding the "autoimmune kaleidoscope".Most cases of chronic urticaria (CU) are considered idiopathic. It has recently been accepted that autoimmunity plays a critical role in the pathogenesis of CU in some of these patients. Although urticaria is common in the pediatric population, the knowledge regarding CU-associated autoimmunity is very limited. We describe the association of CU with a wide spectrum of clinical and laboratory autoimmune disorders in 2 children and emphasize the concept that CU is another manifestation of the "autoimmune kaleidoscope."- - - - - - - - - - ranking = 1keywords = idiopathic (Clic here for more details about this article) |
8/56. Cholinergic urticaria associated with acquired generalized hypohidrosis: report of a case and review of the literature.Acquired generalized hypohidrosis/anhidrosis is a rare condition of unknown pathogenesis, while idiopathic cholinergic urticaria is relatively common. We report the case of a 19-year-old male with cholinergic urticaria and acquired generalized hypohidrosis, and review previously published similar cases of this association.- - - - - - - - - - ranking = 1keywords = idiopathic (Clic here for more details about this article) |
9/56. Chronic urticaria associated with chronic myelomonocytic leukemia.Although chronic urticaria is usually idiopathic, in rare cases it may be a sign of underlying malignancy. We describe the first case of chronic urticaria associated with chronic myelomonocytic leukemia. The urticarial lesions healed successfully with etoposide, an antineoplastic agent. This case demonstrates that cases of chronic urticaria should not to be labeled as idiopathic until diligent evaluation has failed to reveal a cause.- - - - - - - - - - ranking = 2keywords = idiopathic (Clic here for more details about this article) |
10/56. Chronic urticaria and helicobacter pylori.BACKGROUND: Although the clinical manifestations of chronic urticaria (CU) are similar in most patients, a variety of factors should be taken into consideration. In general, the cause of CU cannot be determined in most patients, and it is considered idiopathic. In the past several years, relationships between some patients with CU and hepatitis c or autoimmune thyroid diseases have been established. Similarly, other factors may also be considered as possible causes to explain certain patients with CU. Previously, some patients with CU have had their disease attributed to helicobacter pylori (HP), but the relationship was only clinical. OBJECTIVE: None of the patients previously described included an immunological study. Thus, we studied a patient with CU, who showed marked clinical improvement after eradication of HP, to demonstrate an IgE relationship with this skin disease. methods: First, blood analytical parameters, roentgenograms, fecal examination for parasites, and skin tests were performed to try to establish an etiology. In addition, endoscopy with gastric biopsy confirmed HP colonization, and eradication treatment was prescribed. To investigate an immunological relationship, other tests performed included the following: HP-specific IgG, histamine release induced by HP, HP-specific IgE, and sodium dodecyl sulfate-polyacrylamide gel electrophoresis with immunoblotting. RESULTS: The blood analytical parameters, roentgenograms, fecal examination for parasites, and skin tests were all negative. In contrast, the tests for HP-specific IgG, histamine release induced by HP, and HP-specific IgE were all positive. In addition, the sulfate-polyacrylamide gel electrophoresis with immunoblotting showed specific IgE binding to an extract of HP. CONCLUSIONS: Our results may indicate an immunological IgE relationship between HP colonization and CU in this particular patient.- - - - - - - - - - ranking = 1keywords = idiopathic (Clic here for more details about this article) |
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