Year : 2007 | Volume
: 52 | Issue : 2 | Page : 106--108
Angioedema - a rare cause of acute episodic dysuria: Case report and review of literature
Radhakrishna Panicker, Abdul Rahman Al Duwaisan
Department of Allergy, Allergy Center, Ministry of Health, Kuwait
Al Rashed Allergy Center, P O Box - 31505, Sulaibikhat 90806
The presentations of acute angioedema vary and depend upon the organs involved. Acute episodes of angioedema can be life-threatening if it involves the larynx leading to the obstruction of upper airways and it is a major cause of death. Angioedema involving the lips, cheek and tongue are common with bizarre presentations. Angioedema of the gastro intestinal tract frequently mimic an acute abdomen resulting in unnecessary surgical intervention and increased morbidity. Here we present an unusual case of angioedema involving the external urethra resulting in severe discomfort and burning sensation during micturition. He was later investigated and diagnosed as idiopathic urticaria with angioedema and promptly responded to antihistamines.
|How to cite this article:|
Panicker R, Al Duwaisan AR. Angioedema - a rare cause of acute episodic dysuria: Case report and review of literature.Indian J Dermatol 2007;52:106-108
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Panicker R, Al Duwaisan AR. Angioedema - a rare cause of acute episodic dysuria: Case report and review of literature. Indian J Dermatol [serial online] 2007 [cited 2023 Feb 6 ];52:106-108
Available from: https://www.e-ijd.org/text.asp?2007/52/2/106/33291
Urticaria or hives refers to a skin eruption consisting of raised, demarcated, intensely itchy papules or wheals. Urticaria involves superficial dermis, while angioedema is due to edema in the deeper subcutaneous and submucosal tissues and manifests as recurrent attacks of intense massive localized edema. Angioedema and urticaria should be viewed as manifestations of the same pathologic process. Postcapillary venule inflammation results in fluid leakage and edema in both conditions. Angioedema involves the vessels in the layers of the skin below the dermis. If urticarial rashes have been occurring for less than six weeks condition is classified as acute urticaria. If lesions persist six weeks or more, it is categorized as chronic urticaria. There are many reports in the literature about the various presentations of acute angioedema like acute laryngeal edema with severe respiratory obstruction, intestinal mucosal swelling presenting as acute abdomen. However there are no reports of acute angioedema with severe episodic dysuria.
A 35-year-old Indian male attended the outpatient department of the allergy center in March 2006 with history of swelling of lower lip, erythematous skin rashes, intense pain during micturition of about three weeks duration. The symptoms started as intermittent intense burning sensation of the external genitalia during micturition. It was not associated with fever, pyuria or hematuria. No history of abdominal pain, vomiting or passing any stones per urine. No history of diabetes, hypertension. No history of pain joints, sore throat. It was associated with mild pruritic skin rashes, which he ignored. Physical examination was unremarkable except for urticarial rashes all over the body. He did not have any history of fever, joints pain, no history suggestive cholinergic urticaria. He denied any family history of angioedema, drug or food allergy. External genitalia was normal.
Routine investigations like complete blood count, ESR, urine routine examination, microscopy and urine culture revealed no abnormality. Repeated urine culture and microscopy did not find any organism.
He had received a course of antibiotic empirically for the severe dysurea. The symptoms were persisting despite antibiotics with severe discomfort during micturition, hence he was referred to the urology department. The investigations were repeated with normal results. An abdominal ultra sound, plain X-ray abdomen were normal without any evidence of renal calculi and normal uroflowmetry. This time urticaria became severe with swelling of the lips typical of angioedema. He was treated with antihistamines and intravenous steroids and the rashes and angioedema subsided. Interestingly urinary symptoms were completely subsided after this treatment. Thereafter his micturition was associated with angioedema of the lips and the symptoms readily responded to cetrizine and recurred on withdrawal of cetrizine.
He was investigated for the possible etiology of angioedema and urticaria.
Early recognition of angioedema is important because significant iatrogenic morbidity may result if the diagnosis is not established. The diagnosis is aided by a detailed past and family history, history of allergy to any food, drug, detailed history of environmental factors. Angioedema can be hereditary and acquired. Hereditary angioedema is inherited as an autosomal dominant condition for which there are two forms. One form is manifested by the absence of C1 esterase inhibitor and the other form by normal levels but the enzyme is dysfunctional. The absence of or dysfunction of C1 esterase inhibitor allows uncontrolled activation of complement cascade, resulting in angioedema. Recently an X-linked inherited angioedema has been described and named as type 3 hereditory angioedema.  Acquired forms of angioedema are associated with an underlying hematologic malignancy or immune complex disease, resulting in activation of C1q in the complement cascade. The acquired and hereditary forms can be differentiated from each other by quantification of C1q levels. The c1q level in the hereditary form is normal and the level is low in the acquired form. Urticaria is a common condition that affects 15% to 24% of the US population at some time in their life.  Urticarial lesions can sometimes be difficult to differentiate from urticarial vasculitis. However urticarial vasculitic lesions generally lasts longer than 24h and leave some hyperpigmentation as they resolve. Also urticarial vasculitic lesions tend to be painful and to burn rather than itch. When there is difficulty in differentiating urticaria from vasculitis, a skin biopsy may give a definite answer.
Recommended initial investigations for urticaria and angioedema include screening test for hematological abnormalities, infections, rheumatologic conditions, thyroid dysfunction, hepatic and renal dysfunction. Further tests may be necessary, depending on the patient's history and initial laboratory tests. C1 inhibitor (functional and quantitative), C1q, C4 and CH50, should be pursued. An abnormality of C1 inhibitor and low C4 level indicate hereditary angioedema, a low C1q level would suggest acquired angioedema, in mastocytosis, the tryptase level is elevated. Tests for thyroid antibodies might be considered. If the sedimentation rate is high serologic tests for cryoglobulins, hepatitis B and C should be considered. Despite a complete examination and extensive laboratory studies, a cause of chronic urticaria/angioedema is not usually identified.
Angioedema is associated with little or no pruritus but it is described as painful or burning  Angioedema generally lasts 12-48h and occurs in 40% patients with urticaria.  It commonly involves the lips, eye lids, face, extremeties and genitalia in an asymmetrical manner , Laryngeal swelling is rare when angioedema is associated with urticaria, but it can occur with C1 inhibitor deficiency, angioedema caused by angiotensin converting enzyme (ACE) inhibitors or anaphylaxis , Angioedema can also involve the gastro intestinal tract, and cause abdominal pain, nausea, vomiting and diarrhea. Vantroyen et al. reported a case of angioedema in a 65-year-old woman with recurrent acute abdomen and ascites, who had undergone two negative surgical interventions before establishing the diagnosis  Angioedema without urticaria occur in underlying complement disorder and laryngeal edema is common and a major cause of death.
ACE inhibitors have been associated with occurrences of angioedema that classically involve the tongue.  This class of medication can cause angioedema years after initiation of the treatment. 
Food allergy and insect sting reactions can trigger acute urticaria. Patients experiencing symptoms associated with specific physical stimuli such as cold, exercise, heat or sweating may have a form of physical urticaria. Finally a complete review of systems is important, because results may point to possible auto immune, connective tissue or lymphoproliferative disorders that may be associated with urticaria. Studies suggest that up to 40% of patients with chronic urticaria have a circulating IgG antibody directed against a subunit of the high affinity IgE receptor and an additional 5% to 10% have antibodies against IgE. 
Chronic urticaria has been associated with a number of diseases, especially auto-immune diseases. Of these thyroid disorders are most common. Thyroid auto-antibodies (antithyroglobulins and antimicrosomal antibodies) are found in 24% cases and thyroid dysfunction is found in 19%. , Hashimotos thyroiditis is more common than Graves disease. Other associated conditions that need to be considered include connective tissue disorders, systemic vasculitis, mastocytosis, malignancy (gastrointestinal cancer, lung cancer, lymphoma) infections. Although angioedema involving eye lid, lip, tongue or internal organs like larynx, GI tract etc is reported, angioedema of urethra causing dysuria is never reported. We report this case for its unusual presentation.
|1||Nzeako UC, Frigas E, Trmaine WJ. Hereditary angioedema: A broad review for clinicians. Arch Intern Med 2001;161:2417-29.|
|2||Joint task Force on practice parameters. The diagnosis and management of urticaria: Practice parameter part 1: Acute urticaria/angioedema part II: chronic urticaria/angioedema. Joint Task Force on Practice Parameters. Ann Allergy Asthma Immunol 2000;85:521-44.|
|3||Kaplan AP. Urticaria and angioedema. In : Middleton E Jr, Reed CE, Ellis EF. Allergy: Principles and practice. 6 th ed, vol 2. Mosby-Year Book: St Louis; 2002. p. 1537-58.|
|4||Kaplan AP. Chronic urticaria: Pathogenesis and treatment. J Allergy Clin Immunol 2004;114:465-74.|
|5||Mastalerz L, Setkowicz M, Sanak M, Szczeklik A. Hypersensitivity to aspirin: Common eicosanoid alterations in urticaria and asthma. J Allergy Clin Immunol 2004;113:771-5.|
|6||Vantroyen B, Knockaert DC. Acquired C1-esterase inhibitor deficiency: A rare cause of episodic acute abdominal pain and ascites. Eur J Emerg Med 2003;10:246-9.|
|7||Brown NJ, Ray WA, Snowden M, Griffin MR. Black Americans have an increased rate of angiotensin converting enzyme inhibitor associated angioedema. Clin Pharmacol Ther 1996;60:8-13.|
|8||Cicardi M, Zingale LC, Bergamaschini L, Agostoni A. Angioedema associated with angiotensin-converting enzyme inhibitor use: Outcome after switching to a different treatment. Arch Intern Med 2004;164:910-3.|
|9||Kaplan AP. Clinical practice. Chronic urticaria and angioedema. N Engl J Med 2002;346:175-9.|
|10||Kikuchi Y, Kaplan AP. Mechanisms of autoimmune activation of basophils in chronic urticaria. J All Clin Immunol 2001;110:492-9.|