Isolated Angioedema is quite common in middle aged post-menopausal women and initially presents with facial swelling (eyelid, lip and tongue) and then may progress to affect other soft tissues in the throat, armpits and genital area. Tongue and laryngeal angioedema are potentially life-threatening . Many cases are not associated with urticaria or hives and on testing, no obvious cause is ever found.
A low level complement C4 (less than 12) in the blood is a good screening test for the Hereditary form of Angioedema (where there is usually a family history of facial swelling and deficiency of C1 Esterase Inhibitor in the Complement system). Salicylic acid, aspirin and the non –steroidal anti-inflammatory medications such as ibuprofen and diclofenac are another potential triggers for isolated angioedema as are the first generation ACE inhibitor blood pressure medications such as ramipril, perindopril, enalapril and lisinopril, which cause accumulation of bradykinin pro-inflammatory mediators.
Angioedema can be further be subdivided into “Histaminergic” Angioedema (cases that respond promptly to antihistamines and oral steroids) and “Non –Histaminergic” Angioedema (those reculcitrant cases that do not respond to antihistamine and steroid medication). The Non-Histaminergic patients can be very difficult to treat, and often have to go onto androgenic (male) hormones such as danazol, stranazol but may occasionally respond to pro-clotting Tranexamic acid (also use to treat heavy periods).
A recent published medical study has shown a very impressive response to progestagen hormones such as noresthisterone and even the “progesterone only” oral contraceptive. A single dose of Depo Provera injected every 3 months was effective in controlling angioedema in 82% of study cases – which is a phenomenal response for this troublesome and potentially life-threatening condition.
Reference: Saule C, Boccon-Gibod I, Fain O et al. Benefits of progestin contracepion in non-allergic angioedema. Clinical and Experimental Allergy April 2013
