Introduction Angioedema is triggered by deep dermal, subcutaneous and submucosal vascular leakage that results in visible, nonpitting swelling. Subacute or chronic facial edema may be due to histamine or bradykinin-mediated angioedema, but alternate causes should be considered when symptoms are atypical or unresponsive to therapy. Case Description We evaluated a 24-year-old male with history of severe aplastic anemia, status-post unrelated donor allo-transplant and chronic graft-versus-host disease. Thirteen months after transplant while taking chronic prednisone, valacyclovir, voriconazole, pentamadine and amoxicillin prophylaxis, he developed acute worsening of facial edema on a background of chronic, mild generalized anasarca. The facial edema was diffuse with prominent bilateral, symmetric, non-pitting periorbital edema and accompanied non-pruritic flushing. Treatments included intravenous steroids, antihistamines, trials of furosomide and albumin, however this provided minimal relief. The swelling persisted for 12 weeks and progressed in severity, thus all antibiotics were discontinued. The patient was referred to Allergy/Immunology for evaluation of possible drug allergy. On Allergy/Immunology evaluation, despite discontinuation of antibiotics, notable facial swelling persisted. The patient denied urticaria but reported new onset progressive exertional dyspnea and orthopnea. Physical exam demonstrated diffusely edematous face with significant bilateral periorbital swelling, scattered blisters bilateral forearms, 2+ pitting edema to mid-thighs bilaterally. Serum tryptase and complement levels were normal. CT chest revealed alarge pericardial effusion with echocardiographic confirmation of pericardial tamponade, requiring urgent pericardial drain. The patient's swelling and dyspnea resolved post procedure. Discussion In the setting of chronic atypical facial edema, cardiac and vascular dysfunction should be included in the differential diagnosis. Angioedema is triggered by deep dermal, subcutaneous and submucosal vascular leakage that results in visible, nonpitting swelling. Subacute or chronic facial edema may be due to histamine or bradykinin-mediated angioedema, but alternate causes should be considered when symptoms are atypical or unresponsive to therapy. We evaluated a 24-year-old male with history of severe aplastic anemia, status-post unrelated donor allo-transplant and chronic graft-versus-host disease. Thirteen months after transplant while taking chronic prednisone, valacyclovir, voriconazole, pentamadine and amoxicillin prophylaxis, he developed acute worsening of facial edema on a background of chronic, mild generalized anasarca. The facial edema was diffuse with prominent bilateral, symmetric, non-pitting periorbital edema and accompanied non-pruritic flushing. Treatments included intravenous steroids, antihistamines, trials of furosomide and albumin, however this provided minimal relief. The swelling persisted for 12 weeks and progressed in severity, thus all antibiotics were discontinued. The patient was referred to Allergy/Immunology for evaluation of possible drug allergy. On Allergy/Immunology evaluation, despite discontinuation of antibiotics, notable facial swelling persisted. The patient denied urticaria but reported new onset progressive exertional dyspnea and orthopnea. Physical exam demonstrated diffusely edematous face with significant bilateral periorbital swelling, scattered blisters bilateral forearms, 2+ pitting edema to mid-thighs bilaterally. Serum tryptase and complement levels were normal. CT chest revealed alarge pericardial effusion with echocardiographic confirmation of pericardial tamponade, requiring urgent pericardial drain. The patient's swelling and dyspnea resolved post procedure. In the setting of chronic atypical facial edema, cardiac and vascular dysfunction should be included in the differential diagnosis.
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Jan 1, 2019
Tomás Ortiz-Basso + 6
Open Access