Angioedema
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In medicine and immunology, angioedema is "swelling involving the deep dermis, subcutaneous, or submucosal tissues, representing localized edema. Angioedema often occurs in the face, lips, tongue, and larynx."[1]
Classification
Hereditary angioedema (Hereditary C1 esterase inhibitor deficiency)
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Acquired deficiency is characterized by low levels of complement C1q.
Acquired angioedema (Acquired C1 esterase inhibitor deficiency)
"Acquired C1 esterase inhibitor deficiency is a rare condition associated with autoimmune or low-grade lymphoproliferative disorder."[2]
- Type I disease is associated with lymphoproliferative disorders.[2]
- Type II disease is associated with autoantibodies[2] and monoclonal gammopathies[3].
Acquired deficiency is characterized by low levels of complement C1q.
Diagnosis
Angioedema due to deficiency of complement C1 inhibitor protein may manifest reduced d-dimer levels, especially during attacks.[4]
References
- ↑ Anonymous (2024), Angioedema (English). Medical Subject Headings. U.S. National Library of Medicine.
- ↑ 2.0 2.1 2.2 Markovic SN, Inwards DJ, Frigas EA, Phyliky RP (January 2000). "Acquired C1 esterase inhibitor deficiency". Ann. Intern. Med. 132 (2): 144–50. PMID 10644276. [e]
- ↑ Frémeaux-Bacchi V, Guinnepain MT, Cacoub P, et al (August 2002). "Prevalence of monoclonal gammopathy in patients presenting with acquired angioedema type 2". Am. J. Med. 113 (3): 194–9. PMID 12208377. [e]
- ↑ Cugno M, Zanichelli A, Bellatorre AG, Griffini S, Cicardi M (February 2009). "Plasma biomarkers of acute attacks in patients with angioedema due to C1-inhibitor deficiency". Allergy 64 (2): 254–7. DOI:10.1111/j.1398-9995.2008.01859.x. PMID 19076541. Research Blogging.