angioneurotic edema

A prescription came in to pharmacy electronically from an urgent care for a depo-Medrol injection with an icd10 code for angioneurotic edema

Angioneurotic Edema: a hereditary/genetic form of angioedema, also known as Quinke's disease

• genetic deficiency of C1 esterase inhibitor protein
• autosomal dominant inhereitance
• normally this protein prevents activation of a cascade leading to angioedema
• diagnosis suspected with severe recurrent angioedema
• most common areas: limbs, face, intestinal tract, and airway
• when occurs in larynx --> airway can be compromised
• when occurs in intestinal tract --> severe abdominal pain, nausea, vomiting 
• other symptoms may include fever, joint pain, mucus membrane swelling, brain swelling
• 1/3 of people with condition develop a non-itchy rash called erythema marginatum
• confirm diagnosis by low levels of C1 esterase inhibitor in blood
• attacks can occur every 1-2 weeks and last 3-4 days long
• can be triggered by minor trauma, stress, physical activity, certain medications (anti-hypertensives and heart failure drugs) but can also occur without a known trigger
• treatment & prevention options:
• antihistamines will not work
• androgen steroids may prevent
• C1 esterase inhibitor concentrate prevent attacks
• Cinryze, Ruconest, Berinert -- injected into veins
• encallantide (Kalbitor), icatibant (firazyr) -- injected under skin

There really isn't much evidence in the literature about using steroids for this condition. In fact, the literature I found stated that corticosteroids would not work. Androgen steroids such as danazol, oxandrolone, or methyltestostorone

• these agents work by increasing the level of aminopeptidase P which inactivates kinins which are responsible for angioedema