74 Hereditary angioedema revealed by compartment syndrome

BACKGROUND: Hereditary angioedema (HAE) can present with a wide spectrum of clinical symptoms, including articular features like the compartment syndrome. OBJECTIVE: We present the peculiar case of a young female patient suffering from this rare complement system disorder and presenting as a « wrist oedema ». METHODS: A 14-year-old girl was admitted in the emergency room during a night shift. She presented with acute compartment (wrist) syndrome due to edematous compression of the median nerve, associated with a typical personal history of HAE. It resolved within 6 h with IV steroids and hyperhydration. The quantitative and qualitative C1 INH esterase returning negative, the diagnosis of HAE type III was made. The patient was treated with long-term treatment based on Danazol, (the most frequently prescribed attenuated androgen to treat HAE). RESULTS: HAE is due to the deficiency or dysfunction of the C1 inhibitor protein (quantitative or qualitative deficiency). A new nomenclature has replaced the initial use of denominations HAE type 1, 2 or 3; to speak rather of HAE with a deficient C1 inhibitor (type 1), with a dysfunctional C1 inhibitor (type 2) or with a normal C1 inhibitor (type 3) Type 3 HAE is rare and difficult to treat. It may respond to anti-estrogenic drugs such as progestins and especially to androgens; specific (very expensive) molecules do exist and act directly on the bradykinin pathways CONCLUSION: A compartment syndrome may reveal extremely rare conditions such as hereditary angioedema. Early recognition and management are the guarantee of a preserved functional prognosis.