A Patient Referred for Evaluation of Angioedema

CASE REPORT The patient is a 43-year-old black female who was born and resides in the Virgin Islands. She had traveled to visit friends in the United States. While in the United States she consulted a dermatologist complaining of facial swelling, and was subsequently referred for evaluation of angioedema. The patient reported a 15-month history of facial swelling. Initially she experienced left periorbital swelling which varied in intensity, but could be severe leading to total closure of the eye. There was mild associated erythema. She tried diphenhydramine with marginal, if any, improvement. She had episodes monthly for 4 months. In the fifth month of her disease she developed swelling on the right side of her face involving the right periorbital area, the right cheek, and the right ear. She used diphenhydramine again, and was also given a 10-day course of amoxicillin, with no improvement. She had several episodes over the next 3 months, each lasting approximately 7 days. In the ninth month she developed bilateral periorbital and paranasal swelling, and this persisted until presentation. The patient denied associated pain, pruritus, or burning sensation with the swelling. Around this same time the patient began to develop other problems. The patient developed a rash, described as red areas with “bumps” which affected the chest, back, arms, and legs. She developed darkening of the skin of her forehead which persisted to presentation. She also developed hair loss and pruritus of the scalp. Other complaints included lesions on her fingers. She described them as small “callouses” on the sides of her fingers. She also complained of thickened skin on her periungual areas that was tender to touch. She also complained of muscle pain and soreness upon movement in the shoulder, upper arm, and upper leg areas. She noted difficulty in climbing stairs. She denied lip, tongue, or throat swelling. She denied dyspnea or wheezing. She denied abdominal complaints, including no diarrhea, and denied dizziness or episodes of syncope. She was on no regular medications, neither was she taking an angiotensinconverting enzyme inhibitor. She denied taking aspirin or other non-steroidal anti-inflammatory medications. She had no history of drug allergies. She had no history of food allergy and could not relate any food as a problem. She denied insect stings. She had no family history of any similar condition. She wore latex gloves at work on a regular basis but denied any worsening of symptoms around latex. Further, her problems began during a 3-month absence from work when she was not exposed to latex. On presentation she related the above history and was still experiencing facial swelling and erythema; rash on the chest, back, arms, and legs; finger lesions and periungual tenderness; and muscle pain in her shoulders, upper arms, and upper legs. PHYSICAL EXAMINATION On physical examination her respiratory rate and heart rate were normal. Her skin examination revealed darkened, thickened skin on her forehead. She had mild periorbital swelling and erythema. She also had more pronounced paranasal swelling and erythema in a malar distribution. She had a fine, erythematous maculopapular eruption on localized portions of her upper back, arms, and legs. On head and neck examination she had no conjunctival injection. Her throat was clear with no erythema or exudate. Her thyroid examination revealed no enlargement or masses. Her neck and chest wall had no swelling. Her lung fields were clear with no wheezing and she had a regular rate and rhythm with no rub or extra heart sounds. Her abdomen was not distended and there was no tenderness or mass. Her extremities revealed no edema. Her fingers had 3-mm to 4-mm papules which were yellow and firm. Her fingers also revealed scaling, erythematous areas. Her periungual areas were thickened and tender to palpation. Her musculoskeletal examination revealed a slight decrease in strength (4/5) in the upper legs, but otherwise normal strength (5/5). Her neurologic examination was intact with normal cranial nerve responses, reflexes, coordination, and gait.