Cases reported "Urticaria"

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1/6. Pruritic urticarial papules and plaques of pregnancy (PUPPP)--a case report.

    Pruritic urticarial papules and plaques of pregnancy (PUPPP) is a rare dermatosis of unknown etiology that is most frequently seen in primiparas and twin/multiple pregnancies. The prognosis is favorable. We report a case of PUPPP in a primipara and review the clinical signs, differential diagnosis, possible etiologic factors, diagnosis, and therapy.
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2/6. Pruritic urticarial papules and plaques of pregnancy with unusual and extensive palmoplantar involvement.

    BACKGROUND: Pruritic urticarial papules and plaques of pregnancy (PUPPP) is a specific dermatosis of pregnancy common to primigravid women in the third trimester. The rash usually begins on the lower abdomen within striae and spreads to the proximal extremities. Involvement of face, palms, and soles is unusual. Although intensely pruritic, the fetus is unaffected, and the condition does not usually recur. It can be difficult to distinguish PUPPP from pemphigoid gestationis, an autoimmune bullous disorder with potential fetal consequences that may recur with subsequent pregnancy, menses, or hormonal therapy. CASE: A young secundagravida at 36 weeks of gestation with monochorionic twins presented with a 3-week history of a pruritic papular eruption that began on the abdomen and spread to the extremities. She had extensive involvement of the distal extremities, including the palmoplantar surfaces, with small vesicles of 2-4 mm on acral skin. Because of her unusual presentation, she was thought initially to have pemphigoid gestationis. Subsequent dermatological evaluation and a biopsy confirmed the diagnosis of PUPPP. Shortly after admission she delivered 2 healthy male infants, and her rash cleared with conservative management. CONCLUSION: Pruritic urticarial papules and plaques of pregnancy often, but not always, spares the face, palms, and soles. Small vesicles can occur in PUPPP, but formation of true bullae is not observed. Careful dermatological examination and cutaneous biopsy can assist in differentiating PUPPP from pemphigoid gestationis, which is essential for treatment and prognosis.
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3/6. Hypocomplementemic urticarial vasculitis: a rare presentation of systemic lupus erythematosus.

    BACKGROUND: Urticarial vasculitis is a small-vessel vasculitis, presenting clinically as persistent urticarial skin lesions and microscopically as leucocytoclastic vasculitis. Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a distinct type of urticarial vasculitis with multiorgan involvement, whose etiology and link with other diseases are still unknown. Some authors have suggested that HUVS can be accompanied by systemic lupus erythematosus (SLE), and others believe that it is a rare subtype of SLE. Urticarial vasculitis is seen in 7-8% of SLE, while 50% of HUVS patients are diagnosed with SLE. OBSERVATIONS AND RESULTS: We report a case of HUVS associated with SLE with fatal outcome unresponsive to the combination of systemic corticosteroids and azathioprine. CONCLUSIONS: SLE and HUVS share both clinical and laboratory features and are probably not separate entities. It is mostly likely that HUVS and SLE fall into the same spectrum of autoimmune diseases. HUVS is probably a subset of SLE. As both diseases can fatally, it should be kept in mind that the overlap of SLE and HUVS may exhibit a relatively rapid progression and poor prognosis.
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4/6. Angioimmunoblastic lymphadenopathy in a child with unusual clinical and immunologic features.

    We report a case of angioimmunoblastic lymphadenopathy in a child followed for 13 years. Unusual features include prolonged course, cold urticaria, nonthrombocytopenic purpura, poor wound healing, transfusion reactions, and possible neurologic involvement with cerebritis and epileptic seizures. The patient's serum contained a monoclonal cryoglobulin, immunoglobulin g, kappa light chain type, that activated the classic complement pathway in vitro and mediated passive transfer of the cold urticaria. The patient responded well to corticosteroids and has been in clinical remission for 8 years without specific treatment. There is immunologic evidence of persistent residual disease activity. This case illustrates the remarkable diversity of clinical and immunologic features and the variable prognosis of this disorder.
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5/6. Leukocytoclastic vasculitis in subacute cutaneous lupus erythematosus: clinicopathologic study of three cases and review of the literature.

    BACKGROUND: Leukocytoclastic vasculitis associated with subacute cutaneous lupus erythematosus (SCLE) was observed by Sontheimer et al. in their first clinical series of patients with SCLE, although recent reports have suggested that its frequency was rare. OBJECTIVE: To evaluate the prevalence of cutaneous leukocytoclastic vasculitis (CLV) in patients with SCLE and to describe the clinicopathologic manifestations, response to treatment and prognosis of this subgroup of patients. methods: We reviewed all cases of SCLE seen at the Hospital de la Princesa from 1980 to 1995. RESULTS: Three (9%) of our 27 patients with SCLE exhibited CLV and SCLE. purpura, maculoerythematous lesions, urticaria, nodules and necrotic lesions on the leg, trunk and palmoplantar regions were present in our patients with CLV and SCLE. Histologically, SCLE-associated CLV was a small-sized vessel leukocytoclastic vasculitis with intravascular thrombosis in the deep dermis and coexistence of lesions of CLV and SCLE. CONCLUSION: In our patients, CLV was self-limited and not associated with a worsened prognosis, with only cutaneous involvement in their further relapses of SCLE.
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6/6. Idiopathic anaphylaxis.

    Three cases of idiopathic anaphylaxis are presented. This condition represents a rare but important cause of collapse. The development of atrial fibrillation, reported here for the first time, may lead to diagnostic difficulties. Early recognition and treatment is essential and will result in a good prognosis.
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