Chest
Volume 133, Issue 3, March 2008, Pages 787-789
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SELECTED REPORTS
A Case of Precocious Emphysema and Lung Cancer in a Woman With a History of Hypocomplementemic Urticarial Vasculitis

https://doi.org/10.1378/chest.07-0942Get rights and content

Severe emphysema developed in a white woman with a 26–pack-year history of tobacco use. Serum α1-antitrypsin levels were normal. A history of autoimmune hemolytic anemia, angioedema, low complement, and recurrent urticaria prompted an immunologic workup that ultimately led to a diagnosis of hypocomplementemic urticarial vasculitis syndrome. Treatment with oral prednisone and inhaled bronchodilators improved symptoms, but 4 months after diagnosis non-small cell lung cancer was discovered and she ultimately died. Hypocomplementemic urticarial vasculitis is an uncommon cause of precocious emphysema and has not previously been reported in a patient with bronchogenic carcinoma.

Section snippets

Case Report

A 41-year-old woman presented to the clinic with a 7-year history of progressive dyspnea. She had no cough or mucous production but did occasionally have wheezing.

Her medical history was significant for recurrent episodes of urticaria, angioedema, and arthralgias beginning at age 30 years. A single episode of autoimmune hemolytic anemia and an intermediate antinuclear antibody titer prompted consideration of a diagnosis of systemic lupus erythematosus (SLE). Over time, clinical criteria for

Discussion

McDuffie and colleagues1 first described the uncommon entity of hypocomplementemic urticarial vasculitis syndrome (HUVS) in 1973. They suggested that in order to establish the diagnosis that patients have two major criteria: urticaria of > 6 months in duration and hypocomplementemia; plus two minor criteria: dermal venulitis by biopsy, arthralgia or arthritis, glomerulonephritis, uveitis or episcleritis, recurrent abdominal pain, and a positive C1q precipitin test result. Additional clinical

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  • Cited by (16)

    • Hypocomplementemic urticarial vasculitis

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    • Urticarial lesions: If not urticaria, what else? The differential diagnosis of urticaria. Part II. Systemic diseases

      2010, Journal of the American Academy of Dermatology
      Citation Excerpt :

      Among these, the most common are autoimmune connective tissue diseases (particularly SLE,19,20,48-55 but also systemic sclerosis,56 Gougerot–Sjögren syndrome,57 and paraneoplastic dermatomyositis58), followed by infections (mainly chronic or acute viral hepatitis59-64 and sporadic reports of Epstein–Barr virus,65 Mycoplasma pneumoniae,66 and Lyme disease67) and inflammatory bowel diseases.68 In some cases, UV can be a paraneoplastic manifestation, mainly associated with hematologic malignancies, including mostly non-Hodgkin B-cell lymphomas,69-71 monoclonal gammopathies/myeloma,72-74 anecdotal cases of Castleman disease,75 and polycythemia rubra vera,76 but also with various solid neoplasms (non–small cell lung cancer,77 renal cancer,78 testicular teratoma,79 colon adenocarcinoma,80 and nasopharyngeal carcinoma58). Finally, the vasculitic process that causes skin lesions can also involve internal organs, particularly the joints, kidneys, and lungs, and also the gastrointestinal tract and central and peripheral nervous systems.

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    The authors have no conflicts of interest to disclose.

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