Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0042109 (urticaria)
 6,569 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Hereditary periodic fever syndromes are a group of systemic disorders characterized by recurrent attacks of systemic inflammation (autoinflammation) without infectious or autoimmune cause. The hyper-IgD syndrome (HIDS) is a rare autosomal recessive inflammatory disorder characterized by recurrent fever, increased serum IgD (normal value < 100 U/ml) and generalized inflammation (lymphadenopathy, arthralgias/arthritis, abdominal complaints, skin rash, and headache). The attacks persist during the entire life although frequency and severity tend to diminish with age. HIDS is caused by specific mutations in the gene encoding mevalonate kinase, resulting in depressed enzymatic activity. At present the therapy for the syndrome is only supportive. Other than HIDS, other hereditary systemic inflammatory disorders have been described: the Familial Mediterranean Fever, the tumour necrosis factor receptor associated periodic syndrome (TRAPS), a disease related to the mutations of one of the TNF receptors, the Familial Cold Urticaria and the Muckle-Wells syndrome. The differential diagnosis with other causes of periodic fever is crucial for assessing appropriate management and treatment.
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PMID:[Hyper-IgD syndrome and other hereditary periodic fever syndromes]. 1547 May 20

This reports the first 2 cases of serum sicknesslike reaction to bupropion in children (age 12 and 14). Serum sicknesslike reactions are an example of immune-complex medicated disease. The cardinal symptoms of serum sickness are fever, lymphadenopathy, arthralgias or arthritis, and urticaria. Symptoms usually resolve without long-term sequela following discontinuation of the exogenous antigen. It is likely that serum sicknesslike reactions to bupropion are either relatively rare or underrecognized and underreported. Between May 1998 and May 2001, GlaxoSmith Kline received 172 reports of seizures (a well-known adverse drug reaction) and only 37 reports of serum sicknesslike reactions (Wooltorton 2002). We do not know if children and adolescents are more prone than adults to develop serum sicknesslike reactions to bupropion. Luckily, the reported cases of serum sicknesslike reactions to bupropion have not caused irreversible morbidity or mortality. Nevertheless, the symptoms are painful, temporarily disfiguring and disabling, and warrant prompt medical attention. Parents and patients should be educated about this potential side effect at the onset of treatment, because symptoms are similar to many infectious childhood illnesses, and the treatment of serum sicknesslike reactions to bupropion should include the discontinuation of bupropion.
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PMID:Pediatric bupropion-induced serum sicknesslike reaction. 1565 May 6

Chronic lymphocytic leukemia (CLL) is commonly treated with the alkylating agent chlorambucil. Allergic reactions to this chemotherapy are rare. Previous reports include urticaria, angioedema, rashes, toxic epidermal necrolysis, drug fever and one case of immune hemolytic anemia. We report 2 cases that had the identical symptoms of acute onset of high fever and progressive lymphadenopathy. These symptoms disappeared with conservative management. Neither of the patients were treated for infection or disease progression. When the patients were rechallenged with chlorambucil, identical reactions recurred. There was no cross-reactivity with the alkylating agent cyclophosphamide or other types of chemotherapy. This type of reaction to chlorambucil has not been described previously. It is important to recognize this as an allergic reaction. These reactions could be confused with the onset of infection, progression of disease or even a Richter's transformation.
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PMID:Allergic reaction to chlorambucil in chronic lymphocytic leukemia presenting with fever and lymphadenopathy. 1608 62

Schnitzler syndrome is characterized by monoclonal IgM gammopathy, urticaria, recurrent fever, evidence of inflammation, bone pain, and arthralgia, occasionally in combination with lymphadenopathy and/or hepatosplenomegaly. Only about 80 cases have been reported to date. Development of a hematological malignancy is the main complication. Recent reports of remissions induced by IL-1 receptor antagonist therapy shed new light on the pathophysiology of the disease.
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PMID:Schnitzler syndrome. 1837 80

Autoinflammatory diseases are a group of monogenic inflammatory diseases with an early onset in childhood. Previously these diseases were summarized as"periodic fever syndromes." Included in this spectrum are familial Mediterranean fever, mevalonate kinase deficiency, and tumor necrosis factor receptor-associated disease. They are characterized by periodic or recurrent episodes of systemic inflammation causing fever, accompanied by rash, serositis, lymphadenopathy, arthritis, and other clinical manifestations. The other large group of autoinflammatory diseases consists of the cryopyrin-associated periodic syndromes, which include the cryopyrinopathies. The mildest form is familial cold-associated syndrome, a more severe form is Muckle-Wells syndrome, and the most severe is neonatal-onset multisystem inflammatory disease/chronic infantile neurological cutaneous and articular syndrome. These are characterized by chronic or recurrent systemic inflammation associated with various clinical presentations, including urticaria-like rash, arthritis, sensorineural deafness, and central nervous system and bone involvement. In our review we focus on the clinical presentation of these diseases.
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PMID:[Autoinflammatory diseases in childhood]. 1984 24

We report a case of longstanding multidrug resistant Schnitzler's syndrome that finally went into clinical remission upon treatment with anakinra and review the literature concerning IL1-RA treatment for Schnitzler's syndrome. A now 71-year-old patient presenting with recurring episodes of urticaria and fever and secondary weight loss for the past 16 years as well as arthralgia, hearing loss. The patient has anemia, leucocytosis with neutrophilia, thrombocytosis, elevated C-reactive protein and erythrocyte sedimentation rate, lymphadenopathy and a monoclonal IgM kappa band that later became oligoclonal with two IgM kappa bands and one IgM lambda band. The patient was treated with moderate effect with combination of prednisolone, azathioprine, and colchicine. In March 2009, anakinra 100 mg daily sc was added during a disease flare. Within 24 h after the first injection, both the urticaria and the fever disappeared and have not recurred. For the past 6 months, the patient has been in clinical and biochemical remission. Colchicine has been stopped while the azathioprine and prednisolone doses are being reduced. Twenty-four patients with Schnitzler's syndrome, including three patients with a variant of Schnitzler's syndrome and three patients with a Schnitzler-like syndrome, have been successfully treated with anakinra. Nevertheless, seven out of seven patients, that either interrupted or used anakinra every other day, had relapse of their symptoms within 24-48 h; anakinra was restarted in all patients with the same clinical efficiency. The current case history and the literature review already suggest an important role for IL-1 as a mediator in the pathophysiology of Schnitzler's syndrome.
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PMID:Dramatic response to IL1-RA treatment in longstanding multidrug resistant Schnitzler's syndrome: a case report and literature review. 2011 42

The topical immunotherapy is used to treat alopecia areata and recalcitrant warts since the 1970s. Diphencyprone is a contact sensitizer used to treat dermatological conditions resulting from as altered immunological state, such as extensive alopecia areata, being partially effective and safe. Side effects include local eczema with blistering, regional lymphadenopathy and contact urticaria. Rare adverse effects include an erythema multiforme-like reaction, hyperpigmenttion, hypopigmentation, and vitiligo. We report a 30-year-old, Brazilian male who developed vitiligo lesions following DPCP therapy for alopecia areata.
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PMID:Vitiligo after diphencyprone for alopecia areata. 2058 97

Adult-onset Still's disease (AOSD) patients typically present with arthralgia, fever, lymphadenopathy and a transient salmon maculopapular rash. Only approximately 25 cases of AOSD with urticaria were described in the literature. In this article, the authors report three additional cases of AOSD with urticarial and dermographic lesions who had a good clinical response to glucocorticoid and antihistamines. A review of the literature concerning this issue is also herein written.
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PMID:Urticaria and dermographism in patients with adult-onset Still's disease. 2178 58

Brucellosis is a worldwide zoonotic gram-negative bacterium of worldwide distribution. Its role in causing miscarriage in animals is well documented. Data on its role in human abortion are very few. This paper was carried out on selected women with abortion or history of abortion to clarify the role of brucellosis in human abortion. A total of 129 women were selected from Al-Zahraa University Hospital and other obstetric and gynecological hospitals in the vicinity of Greater Cairo. The patients were subjected to clinical, gynecological, and serodiagnosis (STAT and ELISA) of brucellosis. Also, routine urine (Nuclepore technique) and stool (Kato thick smear) was done as well as skin tests and ELISA for common hepatic parasites. The results showed that 59 had brucellosis, 27 had toxoplasmosis, 15 had fascioliasis and 29 had other cause(s) of abortion. Meanwhile, none had visceral leishmaniasis or schistosomiasis mansoni. the signs and symptoms of all patients were hepatosplenomegaly (31.1%), lower back abdominal pain (23.13%), lassitude, headache (each, 21.7%), lymphadenopathy (20.1%), vomiting (17.1%), loss of appetite, myalgia or diarrhea or constipation (each, 15.42 %), weight loss (14.6%), chest pain (13.9%), night sweating or dizziness (11.65%), fever or right sided abdominal pain (each, 10.7%), chills (7.71%), urticaria or monoarthralgia (each, 3.85%). These signs and symptoms were confusing for specific clinical picture of brucellosis. Brucellosis patients were successfully treated with a combination of Rifampicin 600 mg. once daily and Septrin 800 mg twice daily for 6 weeks. Cure was achieved clinically and serologically. Patients with toxoplasmosis or fascioliasis were also treated with Fasinex and Mirazid respectively. Other parasites were also treated.
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PMID:Maternal brucellosis and human pregnancy. 2198 Jul 85

Allergen immunotherapy is commonly incorporated in the management of allergic rhinoconjunctivitis, allergic asthma, and insect sting hypersensitivity. It is generally safe, but systemic reactions occasionally occur, mainly of the immediate type and rarely of the delayed type. We report a case of a 50-year-old man with allergic rhinoconjunctivitis on immunotherapy for 3 years and then received an injection from another patient's extract. The latter contained a higher concentration of house-dust mite and pollens of grasses, trees, and weeds. It also contained molds that the patient's correct extract did not have. Within half an hour, he developed a systemic reaction that resolved with symptomatic treatment. Two weeks later, he received one-half of his usual immunotherapy dose. Within a week, he developed urticaria, arthralgia, myalgia, fever, and lymphadenopathy. Laboratory abnormalities included leukocytosis, elevated erythrocyte sedimentation rate, hematuria, and elevated liver enzymes. Oral corticosteroid therapy for 3 weeks was ineffective. He developed significant myalgia and apparent mood changes, attributable to corticosteroid intake. After a single plasmapheresis, he felt remarkable improvement within <24 hours. Corticosteroid therapy was gradually withdrawn over 10 weeks without relapse of symptoms. This is a rare case of probable serum sickness after the administration of a wrong allergy immunotherapy extract. However, a causal relationship could not be proven. The response was poor to prolonged corticosteroid therapy but was remarkable to one plasmapheresis.
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PMID:Fever, urticaria, lymphadenopathy, and protracted arthralgia and myalgia resistant to corticosteroid therapy. 2219 94


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