Hereditary Angioedema: Not Just Common Angioedema

Abstract

Hereditary angioedema is one of most challenging problems in field of dermatology in term of correct diagnosis and timely management. We present case of a Thai female aged 47- years-old woman form Saraburi province with history of recurrent swelling of extremities after physical injuries with resolved spontaneously within 24 hours; moreover, history of recurrent events of abdominal pain ensued. Swelling of facial, eyelids, lips, tongue, and genitalia had occurred frequently. Upper airway obstruction also presented and severe enough to incur hypoxic arrest. Her father passed away from upper airway obstruction. Her 13-year-old son also had suffered from recurrent episodes of angioedema and abdominal pain. Her blood samples were investigated. Apart from her first visit, her C4 level is between 11.2-12.7 mg/dl, showing persistently low level (normal C4 complement level: 14-44 mg/dl) regardless of her disease status. Her C1 inhibitor is 7.7 mg/dl (normal C1 inhibitor: 19-37 mg/dl), Her C1 activity is 32 percent. She was diagnosed as hereditary angioedema type 1.

Her management included danazol 200 mg orally on alternate day for prophylaxis, icatibant (bradykinin beta 2 receptor antagonist) during an attack, fresh frozen plasma infusion in term of short course prophylaxis, emergency ID card addressing her underlying disease. Her offspring had been investigated and managed promptly.