Masqueraders and other causes of angioedema and hives

1 Patient Presentation. A 49-year-old woman from Maine presented with the chief complaint

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of intermittent swellings for 24 months, which had been called angioedema and urticaria. At the onset, 2 years before presentation, she awakened with swelling of the left eyelid that lasted for 2 days. She assumed that she had been stung or bitten. One month later she had a similar episode involving the other eyelid. After this she had recurrent, painless swelling, at times pruritic, in various parts of her body. The swellings became more frequent and involved the eyes, cheeks, face, forearms, wrists, and trunk. The swellings usually lasted for 2 to3 days and occurred several times weekly. She also described what sounded like urticaria. On one occasion she had a sensation of “strangling” and was told by an otolaryngologist that she had a swollen throat. Just before presentation she developed marked swelling of the conjunctiva of one eye. As she stated, the “eyeball" itself swelled. The swelling had become increasingly more frequent and she was unable to work for one year and a half before presentation she had seen multiple physicians including allergists. Unsuccessful treatments had included antihistamines systemic steroids (methylprednisolone), immunotherapy for 14 months, a 4-day fast, and an elimination diet. During the 4-day fast she developed a severe outbreak of her swellings and presumed that food was not the cause. The patient was a big-game hunter and although born and raised in the United States, she had traveled extensively, including Africa, India, and South America. On these travels she frequently left the usual tourist areas and went into the “bush”. Nine months before the onset of her initial symptoms, she had spent several weeks in Central Africa. Because of our suspicion of possible parasitic disease, blood for serologies was sent to the Centers for Disease Control in Atlanta, Georgia, and these were negative for leishmaniasis, ascariasis, schistosomiasis and Chagas disease; but strongly positive for filariasis (...) Treatment with diethylcarbamazine was advised. She remained symptom-free for 3 months after a 3-week treatment with diethylcarbamazine. She then had a recurrence of her symptoms, and diethylcarbamazine was given again. After the second course, both the patient’s symptoms and her blood eosinophilia resolved. Discussion. This patient had filariasis (specifically, Loa loa, the filarial “eye worm”) causing angioedema like swelling and possible hives. The patient's description of her symptoms sounded like angioedema and urticaria. One atypical feature was the involvement of her conjunctiva and eyeball. It is unusual for angioedema to cause actual eyeball swelling, although eyelid swelling is common. The reason for the eyeball swelling is that this parasite has a predilection for migrating across the eye; thus, it is called the “eye worm." The swellings are called fugitive swellings or Calabar swellings that are thought to result from an allergic response in the host to antigens released by the parasite migrating subcutaneously. The insect vector for this parasite is the deerfly Chrysops and the disease is endemic in the African equatorial rain forest and its fringe areas. The treatment is diethylcarbamazine, and with the treatment the patient may temporarily get worse and also develop encephalitis, so patients should be observed carefully after institution of treatment. Extraído y adaptado Van Dellen, R. G., Maddox. D. E., Dutta. E. J. (2000) Masqueraders of angioedema and urticaria. Annals of Allergy, Asthma & Immunology.Volume 88(1):pp. 10-14