URTICARIA, ANGIOEDEMA นสพ.อาทิ ต ย์ เชยคาดี AND ANAPHYLAXIS
URTICARIADefinition Urtica in Latin is Nettle rash Urticaria (or hives) irregularly shaped wheal with a blanched center surrounded by a red flare itchy rash consisting of a raised Rapid disappear to normal skin in 1-24 hr. But can appear in other area
ANGIOEDEMADefinition Angioedema is the swelling of deep dermis, subcutaneous, or submucosal tissue due to vascular leakage Pain > itchy Often at Mucous membrane Recovery slower than wheal Duration 72 hr.
ANGIOEDEMA Hereditary Type I Type II Hereditary angioedema with normal C1 INH in woman Acquired Allergic : food, drug, insect venoms, radiocontrast media etc. Idiopathic angioedema Drug : NSAID induced Angioedema associated with idiopathic or autoimmune urticaria Angioedema associated with enzyme-inhibitor-induced Angioedema associated with eosinophilia Angioedema associated with physical urticaria and with cholinergic urticaria
ANGIOEDEMA Acquired Angioedema associated with allergic contact urticaria Angioedema associated with urticarial vasculitis Angioedema associated with infection and infestation Acquired C1 INH deficiency
ANGIOEDEMA Normal or elevated serum complement levels IgE-mediated (atopic, specific antigen, exercise) Induced by physical agents Drug : Aspirin, NSAID, contrast media, opiates, polyanionic antibiotic Low serum complement levels Low C1 INH Genetic (Hereditary C1 INH deficiency; HAE) C1 INH deficiency type I and II Acquired (Acquired C1 INH deficiency; AAE) Lyphoproliferative disorders Anti-C1 INH antibodies Normal C1 INH Serum sickness, blood product reaction Necrotizing vasculitis Dyes : contrast media Idiosyncratic
HEREDITARY ANGIOEDEMA Autosomal dominant with incomplete penetrance. Spontaneous mutations in 50% Diminished C4 between attacks Very low C4 during attacks HAE I Low levels of C1 esterase inhibitor HAE II Dysfunctional C1 INH HAE III (estrogen-dependent angioedema) Normal C1 INH amount and function Normal complement levels
DIFFERENTIAL DIAGNOSIS OF ANGIOEDEMACHFLymphedemaThrombophlebitisErysipelasCellulitisChild abuseNephrotic syndrome
DIFFERENTIAL DIAGNOSIS OF ANGIOEDEMASuperior vena cava syndromeMyxedema from congenital hypothyroidismDermatomyositisSclerodermaParasite Trichinella spiralisAllergic contact dermatitisCrohn’s diseaseMelkersson-Rosenthal syndromeCheilitis granulomatosa
DIAGNOSIS OF URTICARIA/ANGIOEDEMAHistory Time of onset of disease Frequency and Duration of healing Size, shape, area and distribution Urticaria and angioedema Pruritus or pain Family history (atopy) Present illness and past history about allergy Physical stimulation or exercise
DIAGNOSIS OF URTICARIA/ANGIOEDEMAHistory Drug used history Food Smoking Occupation Relati onship to the menstrua l cycle Travel Surgical implantations Insect bite Stress Response to treatment
Urticaria/angioedema superficial deep> 24 hr. < 24 hr. + Urticaria - Urticaria Biopsy Urticaria+angioedema Unknown /phatology cause < 6 WK > 6 WK Vasculitis Abnormal Pressure Drug C1INH test + Yes No History/ Demographism test Delayed HAE AAE Urticaria pressure Vasculitis urticaria Acute urticaria Work up / Physical / Cholinergic Chronic urticaria autoimmune urticaria
MANAGEMENTIdentification and elimination of the underlying cause and/or triggerSymptomatic therapy Drug Cold pack Avoid drug is trigger urticaria (NSAID, morphine, ACE inhibitor, ingredient alcohol )
TREATMENT OF HEREDITARY ANGIOEDEMA Patient education very important; test family No regular medication needed in many cases Prophylactic stanozolol or danozol Fresh frozen plasma before emergency surgery C1 inhibitor Symptomatic treatment during attacks Steroids and antihistamines are NOT effective
SUMMARY OF TREATMENTS FOR C1 ESTERASE INHIBITOR DEFICIENCY
CASE Physical examination V/S BT = 38.5 C , PR 120/min, RR = 20 /min, BP =80/40mmHg BW = 20 kg , Height 155 cm GA : A thai boy with good consciousness, no pallor, no jaundice, no cyanosis Skin : dry and swollen lips, flushing, generalized urticaria rash with facial angioedema Heart : tachycardia wit normal S1 S2, no murmur Lungs : expiratory Wheezing on both lungs, no crepitation Other : unremarkable
CASE Positive finding Fever Tachycardia Hypotension Hx of Drug allery Angioedema Urticaria expiratory Wheezing on both lungs Negative finding No stress No redness of body No brown macule No hx of psychological disorder
CASE Problem list Angioedema with generalize urticaria rash with anaphylaxis Fever with mucous diarrhea
DEFINITION OF ANAPHYLAXIS systemic, immediate hypersensitivity Affects body as a whole Multiple organ systems may be involved Onset generally acute Manifestations vary from mild to fatal immunoglobulin E (IgE)-mediated Anaphylatoid Non – immunoglobulin E (IgE)-mediated
CRITERIA FOR ANAPHYLAXISCriterion 1 – Acute onset of an illness involving the skin, mucous membranes at least one of the following: Respiratory compromise Decreased blood pressure or associated symptoms of end-organ dysfunction
CRITERIA FOR ANAPHYLAXISCriterion 2 – Two or more of the following that occur rapidly after exposure to an allergen that is likely for that patient Involvement of the skin and/or mucous membranes Respiratory compromise Decreased blood pressure or associated symptoms Persistent gastrointestinal symptoms
CRITERIA FOR ANAPHYLAXISCriterion 3 – Decreased blood pressure after exposure of a known allergen for that patient Decreased blood pressure is defined in adults as a systolic BP of less than 90 mmHg or >30% decrease from that patient’s baseline. In infants and children, decreased BP is defined as low systolic BP of less than 70 mmHg from one month up to one year less than (70mmHg + [2 x age]} from one to ten years less than 90 mmHg from 11 to 17 years.
DIFFERENTIAL DIAGNOSIS Acute respiratory failure Status asthmaticus Foreign body aspiration Pulmonary embolism Epiglottitis Non organic disease Panic attack Munchausen’s stridor Vocal cord dysfunction Other Red man syndrome (Vancomycin) Hereditary angioedema
LAB INVESTIGATION Serum tryptase Peak at 60- 90 min > 10 nanogram/ml > 1.4 times or 2 nanogram/ml at 1-2 wk after anaphylaxis Sensitivity 73% specific 98 % In some case are normal but mastocytosis rise Specific – IgE Skin test 6 wk after anaphylaxis Serum specific IgE antibody
TREATMENT Support the airway and ventilation; and Give supplementary oxygen. Intramuscular 1: 1000 (1 mg/ml) adrenaline at a dose of 0.01 mg/kg (0.01 ml/kg) body weight up to a maximum dose of 0.5 mg (0.5 ml) Resuscitate with intravenous saline (20 ml/kg body weight) Bronchodilator Systemic corticosteroid Hydrocortisone (5mg/kg q 6 hr.) Methylprednisolone (1mg/kg q 6 hr.)
TREATMENT Antihistamine Chlorpheniramine 0.1mg/kg q 6 hr. Cimetidine 4mg/kg max 300mg q 8-12 hr. Refractory anaphylaxis in patient used beta -blocker Glucagon 20-30 mcg/kg max 1 mg slow push in 5 min and IV drip 5-15 mcg/min until BP stable Bradycardia Atropine 0.5 mg q 10 min cumulative dose 2 mg
PREVENTION Agents causing anaphylaxis should be identified when possible and avoided Individuals at high risk for anaphylaxis should be issued epinephrine syringes for self -administration and instructed in their use Beta-adrenergic antagonists should be avoided, whenever possible. Children and their care -givers should be offered a written emergency plan in case of accidental ingestion. Pre-treatment with glucocorticosteroids and H1 and H2 antihistamines when used radio contrast media in some case
PREVENTION Patients with egg allergy should be tested before receiving measles, influenza or yellow fever vaccines which contain egg protein. In cases of food-associated exercise-induced anaphylaxis, children must not exercise within 4 hours of ingesting the triggering food Reactions to medications can be reduced and minimized by using oral medications in preference to injected forms. The use of powder-free, low allergen gloves and materials should be used in children undergoing multiple surgeries.
REFERENCE Zuberbier T, Bindslev-Jensen C, Canonica W, Grattan CE, Greaves MW, Henz BM, et al. EAACI/GA2LEN/EDF guideline: definition, classifica tion and diagnosis of urticaria. Allergy 2006; 61:316-20. Zuberbier T, Bindslev-Jensen C, Canonica W, Grattan CE, Greaves MW, Henz BM, et al. EAACI/GA2LEN/EDF guideline: management of urticaria. Allergy 2006; 61:321-31. Grattan CEH, Humphreys. Guidelines for evaluation and management of urticaria in adults and children. Br J Dermatol 2007; 157: 1116-23. M. Scott Linscott, Anaphylaxis: Diagnosis and Management in the Rural Emergency Department. American Journal of Clinical Medicine 2012 ; 91. Donald Y.M. Leung, Stephen C. Dreskin. Urticaria (Hives) and Angioedema. In: Behrman RE, Kliegman RM, Jenson HB. Nelson Textbook of Pediatrics. 18th ed. Philadelphia PA: W.B. Saunders; 2007. Elham Hossny. Anaphylaxis in children. Egypt J Pediatr Allergy Immunol 2007; 5(2): 47-54.