Cases reported "Urticaria"

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1/17. Severe dermographism after topical therapy with diphenylcyclopropenone for alopecia universalis.

    We describe here a 19-year-old Japanese man with an 11-year history of alopecia universalis, who, after the 1st application of a 0.003% diphenylcyclopropenone (DPCP) solution to the whole scalp, developed acute contact dermatitis at the test site, together with widespread severe dermographism. Every 3 weeks, persistence of the severe urticarial reaction and efficacy of treatment were monitored by constant pressure stimuli in a series of pressure tests, and subsequently evaluated by laser Doppler flowmetry (LDF). Although, on pressure tests, the urticarial response was found to significantly improve after starting treatment, erythematous responses continued to appear for nearly 3 months. The persistent course of these side-effects in our patient strongly suggests that precautions must currently be taken in the therapeutic use of potent sensitizers such as DPCP.
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2/17. Persistent urticaria--urticarial reaction caused by late phase reaction?

    In ordinary urticaria, individual lesions disappear within 24 hours. We encountered 3 patients who showed urticarial reactions lasting more than 24 hours. In all patients, skin biopsy revealed interstitial dermal edema and a perivascular infiltration predominated by eosinophils, without immunoglobulins or complement deposition, or endothelial fibrinoid degeneration. Their eosinophil counts and serum complement levels were within normal range. No proteinurea or joint pain was observed. They could not be controlled by any medications except for glucocorticoid. These findings indicate our cases are not ordinary urticaria, urticarial reaction accompanied by eosinophilia, urticarial vasculitis or delayed pressure urticaria. We recognize such urticarial reaction as a different clinical entity from the usual urticaria, and we speculate that this condition is caused by late phase reaction because of the clinical course and eosinophil infiltrations.
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3/17. A pruritic linear urticarial rash, fever, and systemic inflammatory disease in five adolescents: adult-onset still disease or systemic juvenile idiopathic arthritis sine arthritis?

    The characteristic rash of systemic juvenile idiopathic arthritis is a transient erythematous eruption associated with a quotidian spiking fever. Usually asymptomatic, it can be pruritic, with dermatographism at sites of scratching or pressure. An illness similar to this entity in adults is designated adult-onset Still disease. The relationship between the pediatric and adult disease is uncertain and differences in case definition have evolved. Specifically, a sustained arthritis for at least 6 weeks is required for a diagnosis of systemic juvenile idiopathic arthritis, whereas transient arthritis and arthralgia are accepted criteria in adult-onset Still disease. We describe five patients less than 16 years of age who presented with an acute illness characterized by fever and a distinctive skin eruption. Intense pruritus and linear erythematous lesions flared with a spiking fever, usually in the late afternoon and evening. Periorbital edema/erythema and nonlinear urticarial lesions were also seen. Two children had splinter hemorrhages of the nail beds and one girl developed a fixed, scaling, pigmented, linear eruption. Severe malaise, myalgia, arthralgia, and leukocytosis were present in every patient. Other systemic manifestations included sore throat, transient arthritis, abdominal pain, lymphadenopathy, hepatomegaly, splenomegaly, hyperferritinemia, and hepatic dysfunction. No patient had a sustained arthritis. The course of the disease was variable. One patient, diagnosed with macrophage activation syndrome, recovered on oral naproxen. Two patients responded to systemic corticosteroid therapy. One girl developed status epilepticus and died from aspiration and asphyxia. A boy with severe hepatitis developed renal failure and thrombotic thrombocytopenic purpura and was treated with plasmapheresis, dialysis, and systemic corticosteroids; he had recurrent episodes of rash and fever into adult life. These children did not fulfill the case definition of systemic juvenile idiopathic arthritis because they lacked a persistent arthritis. adolescent and adult patients with the same clinical and laboratory findings are described under the rubric of adult-onset Still disease. Recognition of the distinctive urticarial skin eruption and spiking fever is important in the diagnosis of a disease with severe morbidity and potentially life-threatening complications.
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4/17. Bullous delayed pressure urticaria: pathogenic role for eosinophilic granulocytes?

    Bullous delayed pressure urticaria (DPU) is a rare variant of DPU. Treatment of DPU is difficult and the underlying pathogenic mechanism of DPU remains elusive. We report a 72-year-old man with DPU and associated chronic urticaria as well as delayed urticarial dermographism. Pressure challenge gave rise to a deep weal covered by multiple vesicles and bullae after 24 h. Histological examination of a skin biopsy specimen obtained 24 h after pressure challenge demonstrated intraepidermal bullae filled with eosinophils accompanied by a dense, predominantly eosinophilic infiltrate in the dermis. Whereas the numbers and morphology of mast cells were unaltered, the extracellular deposition of eosinophil cationic protein revealed evidence for eosinophil activation. Concomitantly, both CD4 and CD8 T lymphocytes were present in the infiltrate and expressed interleukin 5. As bullous DPU may represent the maximal variant of DPU, the investigation of the cellular infiltrate and the chemokines/cytokines released may reveal potential pathogenic mechanisms. A possible effector role of eosinophilic granulocytes, T-cell subsets and mast cells is discussed.
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5/17. A case of fatal biphasic anaphylaxis secondary to multiple stings: adrenalin and/or a longer observation time could have saved the patient?

    We report the case of an atopic male, 76 years old, with post-myocardial infarction ischaemic cardiopathy, arterial hypertension and a history of insect-sting induced large local reactions who died because of a biphasic anaphylaxis subsequent to multiple Vespid stings (about 15). Within approximately ten minutes after the stings he developed urticaria, extended erythema and hypotension (90/60 mmHg), measured by a family member. The objective physical examination by the emergency doctor at the patient's home revealed an orticarioid reaction and erythema of the back and neck, an unaffected respiratory apparatus and CNS, normal pupils, a pulse rate of 74, normal blood pressure ranging from 120/70 to 130/60 mmHg. The patient was administered antihistamine and corticosteroid through parenteral route. During the 45' observation period at the patient's home the urticaria subsided but not to completion. Approximately 40 minutes after the emergency doctor left, the urticaria reoccurred, angioedema of the neck and worsening asthenia developed. The patient died, despite attempts to resuscitate him by the emergency doctor that had been called out again. A post-mortem examination revealed generalised eodema of the lungs, brain, glottis, and bowels due to the severe characteristic systemic compromise of anaphylaxis. The Authors discuss whether an early use of adrenalin and/or a longer observation time could have saved the patient.
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6/17. Immediate-pressure urticaria--a distinct disorder.

    We report a patient who has an immediate wealing response to pressure in the absence of symptomatic dermographism, which we designate 'immediate-pressure urticaria'. Our patient also suffered from cholinergic urticaria and chronic idiopathic urticaria in the past, demonstrating the clustering of urticarias which frequently occurs.
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7/17. Delayed pressure urticaria syndrome: a clinical expression of interleukin 1.

    Study of a patient with the bullous delayed pressure urticaria syndrome showed remarkable congruence of the extra-cutaneous findings and the known effects of interleukin 1: malaise, fever, myalgia, arthralgia, leukocytosis, increased sedimentation rate, and circulating acute phase reactants. As a result of this "clinical assay" for interleukin 1, we conclude that the delayed pressure urticaria syndrome is the clinical expression of interleukin 1, synthesized in the skin as a result of pressure and released into the circulation. Delayed urticaria, mediated by interleukin 1, is to be contrasted with immediate type urticaria, long known to be histamine mediated.
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8/17. Acute synovial fluid eosinophilia associated with delayed pressure urticaria: a role for mast cells?

    We report a case of exercise induced joint effusion with synovial fluid (SF) eosinophilia of 9,540/mm3 in a patient with delayed pressure urticaria. The SF eosinophilia was an acute but transient event associated with some evidence of local complement activation. Histologic assessment revealed a normal synovial membrane but with no detectable intact mast cells. These observations suggest that mast cells and eosinophils acting in concert can cause joint inflammation.
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9/17. Pseudotumour cerebri in association with polyarthritis, urticaria and cryoglobulinemia.

    Pseudotumour cerebri is a disorder characterized by elevated intracranial pressure in the absence of hydrocephalus and intracranial mass lesions. The cause of this disease remains obscure but has been related to diverse underlying conditions. We report a patient with pseudotumour cerebri, polyarthritis, urticaria, hypocomplementemia and cryoglobulinemia. Serial lumbar punctures were unsuccessful in lowering the cerebrospinal fluid (CSF) pressure. A trial of prednisone, 40 mg daily for 4 weeks, resulted in clinical improvement but was ineffective in lowering the CSF pressure. Circulating immune complexes and complement activation may have played a role in the development of increased intracranial pressure. cryoglobulinemia should be added to the list of disorders associated with pseudotumour cerebri.
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10/17. Symptomatic dermographism.

    urticaria is caused by physical factors in almost 12 percent of cases. These factors include pressure. Dermographism is the appearance of whealing and erythema within minutes where skin has been exposed to pressure or mechanical irritation. Symptomatic dermographism is present when "normal" pressures, such as those encountered in the activities of daily living, cause urticaria. Individuals with symptomatic dermographism can be shown to have a lower pressure threshold for the production of dermographism than normal individuals. A case of symptomatic dermographism is presented, and the differential diagnosis is discussed.
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