Fatal Recurrent Angioedema with Systemic Lupus Erythematosus in a Young Female: A Disturbing Decline

Abstract

Recurrent angioedema is a particularly puzzling problem for clinicians worldwide. The cause is often idiopathic with chronic angioedema recurring over weeks to months. Intake of certain drugs and contact with other offending agents can also induce chronic angioedema. In Systemic Lupus Erythematosus (SLE) patients, recurrent angioedema may be due to an acquired deficiency of C1 inhibitor (C1-INH) caused most probably due to formation of antibody against the C1-INH molecule. A 26-year-old female, who had SLE, presented to the Emergency Department with the chief complaint of puffiness of face, swelling of lips and bilateral lower limbs since one day, which occurred following application of aloe vera cream thrice within a duration of 24 hours. Upon investigations, absolute eosinophil count was raised while C4 and C1q levels were reduced. A diagnosis of acquired angioedema was made. The patient was managed with corticosteroids, cyclophosphamide, antibiotics, antihistamines, oxygen support and other supportive measures. However, the patient’s condition declined further and she succumbed on day 5 of admission. Therefore, this report highlights the importance of chronic angioedema in a patient with SLE and the potential lethal outcome that it can result into.

Keywords

• allergic reaction
• autoimmune disease
• malar rash
• oedema