S3B4 POM: Urticaria/Angioedema

What is Urticaria?

"Hives" Erythematous PRURITIC Edematous Wheals BLANCH w/ pressure SYMMETRICALLY distribuetd Usually last ≤4h, Individual Wheal MAY last ~24h

What is Hereditary Angioedema?

*Autosomal Dominant* (WILL see parent w/ condition) Usually seen in patients ≤30 Edema in ≥1 Organs Presentation depends on site of swelling Attacks last 2-5d -> Spontaneous Resolution 3 types: 2-> C1INH related, 1-> Estrogen

What are the THREE types of Hereditary Angioedema?

1: REDUCED C1INH Levels 2: NORMAL C1INCH Levels, DECREASED Function 3: Estrogen related, NO Complement Changes -> To differentiate Type 1 from Type 2, do a Qualitative AND Quantitative C1INH test

What is the most common exogenous cause of angioedema seen in the ER?

ACE-I -> NO associated Urticaria -> Bradykinin. (Also cause of ACE-Cough) Tx: STOP ACE-I

What would you call an Attack of Hereditary Angioedema in a patient without significant history, and LOW C1q levels?

Acquired C1 Inhibitor Deficiency, "Acquired Angioedema" -> Caused by Massive Amounts of immune complex deposition, which depletes C1INH -> Due to underlying B Cell Lymphoproliferative Disease (Chronic Lymphocytic Leukemia, Non-Hodgkin Lymphoma, etc.) Tx: C1INH Concentrate

Compare Acute vs. Chronic Urticaria/Angioedema?

Acute: ≤6w, More likely to find cause, More common in YOUNGER population, +/- Angioedema Chronic: ≥6w, Less likely to find cause, More common in OLDER population, 80% Idiopathic

What are the two types of Idiopathic Angioedema?

Antihistamine Responsive and Unresponsive

What are some Causes of Chronic Urticaria?

Autoimmune Processes Physical: Heat, Cold, Clothing, Premenstrual Urticarial Vasculitis IgG-anti FceR1 or IgG-anti-IgE

How would you r/o IgG-anti FceR1 or IgG-anti-IgE?

Autologous Serum Challenge (Take blood, refine, put serum on patient's skin and look for rxn)

What could you Tx an Acute Attack of HAE with?

Berinert (C1 Esterase Inhibitor) Note: Your patient will be broke forever OR you can use FFP if your facility is lacking this expensive drug. Note, your patient will get worse before better. You should also call Anesthesia for possible intubation.

What could you Prophylax HAE with?

C1 Esterase Inhibitor (Cinryze)

What are some Causes of Acute Urticaria?

Drug: Rx, Herbals, Vitamins Food: Nuts, Milk, Shellfish, Eggs Insect, Latex Allergies Infection: May occur as part of viral prodrome, linger d/t remaining immune complexes Physical: Heat, Cold, Clothing (tight), Premenstrual

What areas are most commonly affected by Angioedema?

Face, Lips, Tongue, Eyelids -> MAY cause life threatening respiratory distress

What is to be remembered about working up HAE?

First: Is there a HISTORY suggestive of HAE? Second: Measure C4 levels If LOW -> Check C1 If C1 LOW -> Type 1 If C1 LOW FUNCTION -> Type 2 Note, C1q will be Normal

How could you Tx Acute Urticaria?

H1 or H2 Antihistamines Tricyclics Xolair

What is the MCC of Mortality in Hereditary Angioedema?

Laryngeal Edema Mean time from onset-death -> 7h

What are some Systemic Diseases that can present at Chronic Idiopathic Urticaria?

Malignancy Thyroid Disease CTiss Dz: SLE, RA, Sjogrens, Waldenstroms Macroglobulinemia

How would you initially Tx Acute Angioedema?

Probably start with Epi (just in case) C1INH Concentrate Diphenhydramine Prednisone H2 Antihistamines

What is Angioedema?

Urticaria-like process occurring in DEEPER tissues NON-pitting, NOT hot, NON-pruritic Benign "Tingling/Pain"