Dr Dylan Prunster1, Dr Peter Meier1, Professor Fiona Wood1, Dr Helen Douglas1
1Fiona Stanley Hospital – State Burns Service of Western Australia , Perth, Australia
Hereditary angioedema (HAE, C1 inhibitor deficiency) is a rare, potentially life-threatening disorder characterized by bouts of cutaneous and submucosal swelling exacerbated by trauma, stress or illness (Busse & Christiansen 2020). The authors present a case of the successful management of superficial facial burns in a patient with Type 1 HA and the management conundrum it presents.
A 30-year-old female with a background of Type I HAE, sustained flash burns to face, bilateral hand and thighs from a flash burn whilst using aerosolised accelerant to start a fire. She had superficial facial burns with some singing of nasal hair. The patient underwent flexible laryngoscopy in the emergency department demonstrating mild oedema to the arytenoids. She was admitted to the ward for strict elevation, airway observation and with the involvement of the immunology team was commenced on an IV infusion of a C1 esterase inhibitor (abatacept®). Though the patient described several episodes of tingling throat no respiratory compromise occurred and she was discharged on day two of admission with immunology and burns follow up.
HA occurs in approximately 1 in 60,000 individuals.(Busse & Christiansen 2020) Prior to effective therapies, HAE was associated with a mortality rate of 30% due to asphyxiation, though more recent series suggest it may still be as high as 13% (Frank 1976; Agostoni et al. 2004). Laryngeal swelling in HA has been found to take a mean of 7 hours to develop. (Bork et al. 2000) The conservative approach with close observation and early involvement of the immunology team prevented any deterioration and significant morbidity in this case.
We present a rare case of facial burn in a patient with HAE successfully treated with elevation and concentrated intravenous C1 inhibitor.
Dr Dylan Prunster is a surgical service registrar working within the West Australian Health Department with a keen interest in Plastic and Reconstructive Surgery. He obtained his primary medical qualification from the University of Western Australia and subsequently undertook internship and residency at Royal Perth Hospital.