Skip to main content
Top
Published in: Forensic Science, Medicine and Pathology 4/2015

01-12-2015 | Case Report

Fatal laryngeal angioedema: a case report and a workup of angioedema in a forensic setting

Authors: Adriana Krizova, Taylor Gardner, D’Arcy L. Little, V. Arcieri-Piersanti, Michael S. Pollanen

Published in: Forensic Science, Medicine and Pathology | Issue 4/2015

Login to get access

Abstract

Angioedema is an episodic swelling of the deep dermis, subcutis, and/or submucosal tissue due to an increase in local vascular permeability. Swelling may involve skin, respiratory, and gastrointestinal tracts. The most commonly involved areas are the periorbital region and the lips. Here we report a case of a fatal laryngeal obstruction due to angioedema likely caused by an angiotensin-converting-enzyme inhibitor. The decedent, a 58-year-old man, was witnessed developing sudden facial swelling and acute respiratory difficulties quickly followed by unresponsiveness. His past medical history suggested that this was his second episode of angioedema without urticaria. Postmortem examination revealed a complete laryngeal obstruction in the absence of infection, neoplasm, or autoimmune disease. Postmortem computed tomography of the head and neck showed a complete obstruction of the upper airway. Based on the current understanding of the pathophysiology of different types of angioedema, we will suggest a workup of angioedema without urticaria in the forensic setting and offer readers resources they can use in their practice.
Literature
1.
go back to reference Zuberbier T, Aberer W, Asero R, Bindslev-Jensen C, Brzoza Z, Canonica GW, et al. The EAACI/GA(2) LEN/EDF/WAO Guideline for the definition, classification, diagnosis, and management of urticaria: the 2013 revision and update. Allergy. 2014;69:868–87.CrossRefPubMed Zuberbier T, Aberer W, Asero R, Bindslev-Jensen C, Brzoza Z, Canonica GW, et al. The EAACI/GA(2) LEN/EDF/WAO Guideline for the definition, classification, diagnosis, and management of urticaria: the 2013 revision and update. Allergy. 2014;69:868–87.CrossRefPubMed
2.
go back to reference Cicardi M, Aberer W, Banerji A, Bas M, Bernstein JA, Bork K, et al. Classification, diagnosis, and approach to treatment for angioedema: consensus report from the hereditary angioedema international working group. Allergy. 2014;69:602–16.CrossRefPubMed Cicardi M, Aberer W, Banerji A, Bas M, Bernstein JA, Bork K, et al. Classification, diagnosis, and approach to treatment for angioedema: consensus report from the hereditary angioedema international working group. Allergy. 2014;69:602–16.CrossRefPubMed
3.
go back to reference McLean-Tooke A, Goulding M, Bundell C, White J, Hollingsworth P. Postmortem serum tryptase levels in anaphylactic and non-anaphylactic deaths. J Clin Pathol. 2014;67:134–8.CrossRefPubMed McLean-Tooke A, Goulding M, Bundell C, White J, Hollingsworth P. Postmortem serum tryptase levels in anaphylactic and non-anaphylactic deaths. J Clin Pathol. 2014;67:134–8.CrossRefPubMed
4.
go back to reference Sampson HA, Mendelson L, Rosen JP. Fatal and near-fatal anaphylactic reactions to food in children and adolescents. N Engl J Med. 1992;327:380–4.CrossRefPubMed Sampson HA, Mendelson L, Rosen JP. Fatal and near-fatal anaphylactic reactions to food in children and adolescents. N Engl J Med. 1992;327:380–4.CrossRefPubMed
5.
go back to reference Lin RY, Schwartz LB, Curry A, Pesola GR, Knight RJ, Lee HS, et al. Histamine and tryptase levels in patients with acute allergic reactions: an emergency department-based study. J Allergy Clin Immunol. 2000;106:65–71.CrossRefPubMed Lin RY, Schwartz LB, Curry A, Pesola GR, Knight RJ, Lee HS, et al. Histamine and tryptase levels in patients with acute allergic reactions: an emergency department-based study. J Allergy Clin Immunol. 2000;106:65–71.CrossRefPubMed
6.
go back to reference Sala-Cunill A, Cardona V, Labrador-Horrillo M, Luengo O, Esteso O, Garriga T, et al. Usefulness and limitations of sequential serum tryptase for the diagnosis of anaphylaxis in 102 patients. Int Arch Allergy Immunol. 2012;160:192–9.CrossRefPubMed Sala-Cunill A, Cardona V, Labrador-Horrillo M, Luengo O, Esteso O, Garriga T, et al. Usefulness and limitations of sequential serum tryptase for the diagnosis of anaphylaxis in 102 patients. Int Arch Allergy Immunol. 2012;160:192–9.CrossRefPubMed
7.
go back to reference Randall B, Butts J, Halsey JF. Elevated postmortem tryptase in the absence of anaphylaxis. J Forensic Sci. 1995;40:208–11.CrossRefPubMed Randall B, Butts J, Halsey JF. Elevated postmortem tryptase in the absence of anaphylaxis. J Forensic Sci. 1995;40:208–11.CrossRefPubMed
8.
go back to reference Edston E, Eriksson O, van Hage M. Mast cell tryptase in postmortem serum-reference values and confounders. Int J Legal Med. 2007;121:275–80.CrossRefPubMed Edston E, Eriksson O, van Hage M. Mast cell tryptase in postmortem serum-reference values and confounders. Int J Legal Med. 2007;121:275–80.CrossRefPubMed
9.
go back to reference Edston E, van Hage-Hamsten M. Mast cell tryptase and haemolysis after trauma. Forensic Sci Int. 2003;131:8–13.CrossRefPubMed Edston E, van Hage-Hamsten M. Mast cell tryptase and haemolysis after trauma. Forensic Sci Int. 2003;131:8–13.CrossRefPubMed
10.
go back to reference Fineschi V, Cecchi R, Centini F, Reattelli LP, Turillazzi E. Immunohistochemical quantification of pulmonary mast-cells and postmortem blood dosages of tryptase and eosinophil cationic protein in 48 heroin-related deaths. Forensic Sci Int. 2001;120:189–94.CrossRefPubMed Fineschi V, Cecchi R, Centini F, Reattelli LP, Turillazzi E. Immunohistochemical quantification of pulmonary mast-cells and postmortem blood dosages of tryptase and eosinophil cationic protein in 48 heroin-related deaths. Forensic Sci Int. 2001;120:189–94.CrossRefPubMed
12.
go back to reference Ogasawara T, Kitagawa Y, Ogawa T, Yamada T, Kawamura Y, Sano K. MR imaging and thermography of facial angioedema: a case report. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2001;92:473–6.CrossRefPubMed Ogasawara T, Kitagawa Y, Ogawa T, Yamada T, Kawamura Y, Sano K. MR imaging and thermography of facial angioedema: a case report. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2001;92:473–6.CrossRefPubMed
13.
go back to reference Bafunno V, Divella C, Sessa F, Tiscia GL, Castellano G, Gesualdo L, et al. De novo homozygous mutation of the C1 inhibitor gene in a patient with hereditary angioedema. J Allergy Clin Immunol. 2013;132:748–50.CrossRefPubMed Bafunno V, Divella C, Sessa F, Tiscia GL, Castellano G, Gesualdo L, et al. De novo homozygous mutation of the C1 inhibitor gene in a patient with hereditary angioedema. J Allergy Clin Immunol. 2013;132:748–50.CrossRefPubMed
14.
go back to reference Blanch A, Roche O, Urrutia I, Gamboa P, Fontán G, López-Trascasa M. First case of homozygous C1 inhibitor deficiency. J Allergy Clin Immunol. 2006;118:1330–5.CrossRefPubMed Blanch A, Roche O, Urrutia I, Gamboa P, Fontán G, López-Trascasa M. First case of homozygous C1 inhibitor deficiency. J Allergy Clin Immunol. 2006;118:1330–5.CrossRefPubMed
15.
go back to reference López-Lera A, Favier B, de la Cruz RM, Garrido S, Drouet C, López-Trascasa M. A new case of homozygous C1-inhibitor deficiency suggests a role for Arg378 in the control of kinin pathway activation. J Allergy Clin Immunol. 2010;126:1307–10.CrossRefPubMed López-Lera A, Favier B, de la Cruz RM, Garrido S, Drouet C, López-Trascasa M. A new case of homozygous C1-inhibitor deficiency suggests a role for Arg378 in the control of kinin pathway activation. J Allergy Clin Immunol. 2010;126:1307–10.CrossRefPubMed
16.
go back to reference Cichon S, Martin L, Hennies HC, Müller F, Van Driessche K, Karpushova A, et al. Increased activity of coagulation factor XII (Hageman factor) causes hereditary angioedema type III. Am J Hum Genet. 2006;79:1098–104.PubMedCentralCrossRefPubMed Cichon S, Martin L, Hennies HC, Müller F, Van Driessche K, Karpushova A, et al. Increased activity of coagulation factor XII (Hageman factor) causes hereditary angioedema type III. Am J Hum Genet. 2006;79:1098–104.PubMedCentralCrossRefPubMed
17.
go back to reference Bork K, Gül D, Hardt J, Dewald G. Hereditary angioedema with normal C1 inhibitor: clinical symptoms and course. Am J Med. 2007;120:987–92.CrossRefPubMed Bork K, Gül D, Hardt J, Dewald G. Hereditary angioedema with normal C1 inhibitor: clinical symptoms and course. Am J Med. 2007;120:987–92.CrossRefPubMed
18.
go back to reference Bork K, Wulff K, Hardt J, Witzke G, Staubach P. Hereditary angioedema caused by missense mutations in the factor XII gene: clinical features, trigger factors, and therapy. J Allergy Clin Immunol. 2009;124:129–34.CrossRefPubMed Bork K, Wulff K, Hardt J, Witzke G, Staubach P. Hereditary angioedema caused by missense mutations in the factor XII gene: clinical features, trigger factors, and therapy. J Allergy Clin Immunol. 2009;124:129–34.CrossRefPubMed
19.
go back to reference Mandle R, Baron C, Roux E, Sundel R, Gelfand J, Aulak K, et al. Acquired C1 inhibitor deficiency as a result of an autoantibody to the reactive center region of C1 inhibitor. J Immunol. 1994;152:4680–5.PubMed Mandle R, Baron C, Roux E, Sundel R, Gelfand J, Aulak K, et al. Acquired C1 inhibitor deficiency as a result of an autoantibody to the reactive center region of C1 inhibitor. J Immunol. 1994;152:4680–5.PubMed
20.
go back to reference Jackson J, Feighery C. Autoantibody-mediated acquired deficiency of C1 inhibitor. N Engl J Med. 1988;318:122–3.CrossRefPubMed Jackson J, Feighery C. Autoantibody-mediated acquired deficiency of C1 inhibitor. N Engl J Med. 1988;318:122–3.CrossRefPubMed
21.
go back to reference Castelli R, Deliliers DL, Zingale LC, Pogliani EM, Cicardi M. Lymphoproliferative disease and acquired C1 inhibitor deficiency. Haematologica. 2007;92:716–8.CrossRefPubMed Castelli R, Deliliers DL, Zingale LC, Pogliani EM, Cicardi M. Lymphoproliferative disease and acquired C1 inhibitor deficiency. Haematologica. 2007;92:716–8.CrossRefPubMed
22.
go back to reference Levi M, Hack CE, van Oers MH. Rituximab-induced elimination of acquired angioedema due to C1-inhibitor deficiency. Am J Med. 2006;119:e3–5.CrossRefPubMed Levi M, Hack CE, van Oers MH. Rituximab-induced elimination of acquired angioedema due to C1-inhibitor deficiency. Am J Med. 2006;119:e3–5.CrossRefPubMed
23.
go back to reference Alsenz J, Bork K, Loos M. Autoantibody-mediated acquired deficiency of C1 inhibitor. N Engl J Med. 1987;316:1360–6.CrossRefPubMed Alsenz J, Bork K, Loos M. Autoantibody-mediated acquired deficiency of C1 inhibitor. N Engl J Med. 1987;316:1360–6.CrossRefPubMed
24.
go back to reference Donaldson VH, Bernstein DI, Wagner CJ, Mitchell BH, Scinto J, Bernstein IL. Angioneurotic edema with acquired C1-inhibitor deficiency and autoantibody to C1-inhibitor: response to plasmapheresis and cytotoxic therapy. J Lab Clin Med. 1992;119:397–406.PubMed Donaldson VH, Bernstein DI, Wagner CJ, Mitchell BH, Scinto J, Bernstein IL. Angioneurotic edema with acquired C1-inhibitor deficiency and autoantibody to C1-inhibitor: response to plasmapheresis and cytotoxic therapy. J Lab Clin Med. 1992;119:397–406.PubMed
25.
go back to reference Branellec A, Bouillet L, Javaud N, Mekinian A, Boccon-Gibod I, Blanchard-Delaunay C, et al. Acquired C1-inhibitor deficiency: 7 patients treated with rituximab. J Clin Immunol. 2012;32:936–41.CrossRefPubMed Branellec A, Bouillet L, Javaud N, Mekinian A, Boccon-Gibod I, Blanchard-Delaunay C, et al. Acquired C1-inhibitor deficiency: 7 patients treated with rituximab. J Clin Immunol. 2012;32:936–41.CrossRefPubMed
27.
go back to reference Sondhi D, Lippmann M, Murali G. Airway compromise due to angiotensin-converting enzyme inhibitor-induced angioedema: clinical experience at a large community teaching hospital. Chest. 2004;126:400–4.CrossRefPubMed Sondhi D, Lippmann M, Murali G. Airway compromise due to angiotensin-converting enzyme inhibitor-induced angioedema: clinical experience at a large community teaching hospital. Chest. 2004;126:400–4.CrossRefPubMed
28.
29.
go back to reference Slater EE, Merrill DD, Guess HA, Roylance PJ, Cooper WD, Inman WH, et al. Clinical profile of angioedema associated with angiotensin converting-enzyme inhibition. JAMA. 1988;260:967–70.CrossRefPubMed Slater EE, Merrill DD, Guess HA, Roylance PJ, Cooper WD, Inman WH, et al. Clinical profile of angioedema associated with angiotensin converting-enzyme inhibition. JAMA. 1988;260:967–70.CrossRefPubMed
30.
go back to reference Guo X, Dick L. Late onset angiotensin-converting enzyme induced angioedema: case report and review of the literature. J Okla State Med Assoc. 1999;92:71–3.PubMed Guo X, Dick L. Late onset angiotensin-converting enzyme induced angioedema: case report and review of the literature. J Okla State Med Assoc. 1999;92:71–3.PubMed
31.
go back to reference Schiller PI, Messmer SL, Haefeli WE, Schlienger RG, Bircher AJ. Angiotensin-converting enzyme inhibitor-induced angioedema: late onset, irregular course, and potential role of triggers. Allergy. 1997;52:432–5.CrossRefPubMed Schiller PI, Messmer SL, Haefeli WE, Schlienger RG, Bircher AJ. Angiotensin-converting enzyme inhibitor-induced angioedema: late onset, irregular course, and potential role of triggers. Allergy. 1997;52:432–5.CrossRefPubMed
32.
go back to reference Agostoni A, Aygören-Pürsün E, Binkley KE, Blanch A, Bork K, Bouillet L, et al. Hereditary and acquired angioedema: problems and progress: proceedings of the third C1 esterase inhibitor deficiency workshop and beyond. J Allergy Clin Immunol. 2004;114:S51–131.CrossRefPubMed Agostoni A, Aygören-Pürsün E, Binkley KE, Blanch A, Bork K, Bouillet L, et al. Hereditary and acquired angioedema: problems and progress: proceedings of the third C1 esterase inhibitor deficiency workshop and beyond. J Allergy Clin Immunol. 2004;114:S51–131.CrossRefPubMed
33.
go back to reference Haymore BR, Yoon J, Mikita CP, Klote MM, DeZee KJ. Risk of angioedema with angiotensin receptor blockers in patients with prior angioedema associated with angiotensin-converting enzyme inhibitors: a meta-analysis. Ann Allergy Asthma Immunol. 2008;101:495–9.CrossRefPubMed Haymore BR, Yoon J, Mikita CP, Klote MM, DeZee KJ. Risk of angioedema with angiotensin receptor blockers in patients with prior angioedema associated with angiotensin-converting enzyme inhibitors: a meta-analysis. Ann Allergy Asthma Immunol. 2008;101:495–9.CrossRefPubMed
34.
35.
go back to reference Lin RY, Cannon AG, Teitel AD. Pattern of hospitalizations for angioedema in New York between 1990 and 2003. Ann Allergy Asthma Immunol. 2005;95:159–66.CrossRefPubMed Lin RY, Cannon AG, Teitel AD. Pattern of hospitalizations for angioedema in New York between 1990 and 2003. Ann Allergy Asthma Immunol. 2005;95:159–66.CrossRefPubMed
36.
go back to reference Dean DE, Schultz DL, Powers RH. Asphyxia due to angiotensin converting enzyme (ACE) inhibitor mediated angioedema of the tongue during the treatment of hypertensive heart disease. J Forensic Sci. 2001;46:1239–43.CrossRefPubMed Dean DE, Schultz DL, Powers RH. Asphyxia due to angiotensin converting enzyme (ACE) inhibitor mediated angioedema of the tongue during the treatment of hypertensive heart disease. J Forensic Sci. 2001;46:1239–43.CrossRefPubMed
37.
go back to reference Cupido C, Rayner B. Life-threatening angio-oedema and death associated with the ACE inhibitor enalapril. S Afr Med J. 2007;97:244–5.PubMed Cupido C, Rayner B. Life-threatening angio-oedema and death associated with the ACE inhibitor enalapril. S Afr Med J. 2007;97:244–5.PubMed
38.
39.
go back to reference Abbosh J, Anderson JA, Levine AB, Kupin WL. Angiotensin converting enzyme inhibitor-induced angioedema more prevalent in transplant patients. Ann Allergy Asthma Immunol. 1999;82:473–6.CrossRefPubMed Abbosh J, Anderson JA, Levine AB, Kupin WL. Angiotensin converting enzyme inhibitor-induced angioedema more prevalent in transplant patients. Ann Allergy Asthma Immunol. 1999;82:473–6.CrossRefPubMed
40.
go back to reference Morimoto T, Gandhi TK, Fiskio JM, Seger AC, So JW, Cook EF, et al. An evaluation of risk factors for adverse drug events associated with angiotensin-converting enzyme inhibitors. J Eval Clin Pract. 2004;10:499–509.CrossRefPubMed Morimoto T, Gandhi TK, Fiskio JM, Seger AC, So JW, Cook EF, et al. An evaluation of risk factors for adverse drug events associated with angiotensin-converting enzyme inhibitors. J Eval Clin Pract. 2004;10:499–509.CrossRefPubMed
41.
go back to reference Kostis JB, Kim HJ, Rusnak J, Casale T, Kaplan A, Corren J, et al. Incidence and characteristics of angioedema associated with enalapril. Arch Intern Med. 2005;165:1637–42.CrossRefPubMed Kostis JB, Kim HJ, Rusnak J, Casale T, Kaplan A, Corren J, et al. Incidence and characteristics of angioedema associated with enalapril. Arch Intern Med. 2005;165:1637–42.CrossRefPubMed
42.
go back to reference Blais C Jr, Marc-Aurèle J, Simmons WH, Loute G, Thibault P, Skidgel RA, et al. Des-Arg9-bradykinin metabolism in patients who presented hypersensitivity reactions during hemodialysis: role of serum ACE and aminopeptidase P. Peptides. 1999;20:421–30.CrossRefPubMed Blais C Jr, Marc-Aurèle J, Simmons WH, Loute G, Thibault P, Skidgel RA, et al. Des-Arg9-bradykinin metabolism in patients who presented hypersensitivity reactions during hemodialysis: role of serum ACE and aminopeptidase P. Peptides. 1999;20:421–30.CrossRefPubMed
43.
go back to reference Cyr M, Hume HA, Champagne M, Sweeney JD, Blais C Jr, Gervais N, et al. Anomaly of the des-Arg9-bradykinin metabolism associated with severe hypotensive reactions during blood transfusions: a preliminary study. Transfusion. 1999;39(10):1084–8.CrossRefPubMed Cyr M, Hume HA, Champagne M, Sweeney JD, Blais C Jr, Gervais N, et al. Anomaly of the des-Arg9-bradykinin metabolism associated with severe hypotensive reactions during blood transfusions: a preliminary study. Transfusion. 1999;39(10):1084–8.CrossRefPubMed
44.
go back to reference Adam A, Cugno M, Molinaro G, Perez M, Lepage Y, Agostoni A. Aminopeptidase P in individuals with a history of angio-oedema on ACE inhibitors. Lancet. 2002;359:2088–9.CrossRefPubMed Adam A, Cugno M, Molinaro G, Perez M, Lepage Y, Agostoni A. Aminopeptidase P in individuals with a history of angio-oedema on ACE inhibitors. Lancet. 2002;359:2088–9.CrossRefPubMed
45.
go back to reference Duan QL, Nikpoor B, Dube MP, Molinaro G, Meijer IA, Dion P, et al. A variant in XPNPEP2 is associated with angioedema induced by angiotensin I-converting enzyme inhibitors. Am J Hum Genet. 2005;77:617–26.PubMedCentralCrossRefPubMed Duan QL, Nikpoor B, Dube MP, Molinaro G, Meijer IA, Dion P, et al. A variant in XPNPEP2 is associated with angioedema induced by angiotensin I-converting enzyme inhibitors. Am J Hum Genet. 2005;77:617–26.PubMedCentralCrossRefPubMed
46.
go back to reference Grover C. Images in clinical medicine. “Thumb Sign” of epiglottitis. N Engl J Med. 2011;365:447.CrossRefPubMed Grover C. Images in clinical medicine. “Thumb Sign” of epiglottitis. N Engl J Med. 2011;365:447.CrossRefPubMed
Metadata
Title
Fatal laryngeal angioedema: a case report and a workup of angioedema in a forensic setting
Authors
Adriana Krizova
Taylor Gardner
D’Arcy L. Little
V. Arcieri-Piersanti
Michael S. Pollanen
Publication date
01-12-2015
Publisher
Springer US
Published in
Forensic Science, Medicine and Pathology / Issue 4/2015
Print ISSN: 1547-769X
Electronic ISSN: 1556-2891
DOI
https://doi.org/10.1007/s12024-015-9701-9

Other articles of this Issue 4/2015

Forensic Science, Medicine and Pathology 4/2015 Go to the issue