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April 2012, Volume 42 Number 4 , p 43 - 46


  • June Pepe LPN, CCRC
  • Leonard Bielory MD


OUR PATIENT, AN, had been diagnosed with hereditary angioedema (HAE) when she was 4 years old. At diagnosis, AN's signs and symptoms were limited to gastrointestinal (GI) attacks and included severe abdominal pain, diarrhea, and vomiting; she'd experienced similar GI problems since the age of 5 months.Her GI attacks had occurred two or three times a year until she started her menstrual cycle, when she began to experience monthly attacks typically lasting for 5 to 7 days. Cutaneous attacks now involved her arms, legs, face, genitals, and periorbital areas, and she experienced laryngeal attacks as well.(See Picturing HAE.)This article explains this rare, life-threatening genetic disorder, including how it's diagnosed and treated. It also describes how new treatment is improving the outcomes for patients with HAE.HAE affects 1 in 50,000 people, according to estimates; the range is reported to be from 1 in 10,000 to 1 in 150,000. Men and women are affected equally. Although patients are usually diagnosed in their second or third decade of life, many patients' symptoms begin earlier. Attacks usually become more frequent after the patient reaches puberty.1 The pattern of signs and symptoms varies considerably between patients and in individual patients. HAE is characterized by recurrent episodes of nonpainful, nonpruritic, nonurticaric angioedema (swelling of deep dermis, subcutaneous, or submucosal tissue due to vascular leakage) that most commonly affects the skin and mucosal tissues of the upper respiratory and GI tracts.1,2 The swelling, which typically develops gradually over several hours, may resolve over 2 to 4 days without treatment. Many patients with HAE have a prodromal cutaneous eruption involving the extremities, chest wall, and abdomen and consisting of a mottled reticulated vascular pattern that appears as an intricate lace-like purplish discoloration. It's similar to livedo reticularis except that it's not limited to the lower extremities. Cutaneous attacks

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