My urticaria in December

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URTICARIA:A chalenging disorder to physicians : 

URTICARIA:A chalenging disorder to physicians Prof. M.YOUSRY ABDEL-MAWLA. Zagazig Faculty of Medicine,EGYPT

DEFINITION : 

M.Y.A.Mawla ZAGAZIG DEFINITION Recurrent attacks of itchy ,short lived ,reddish,evansent swellings (WHEALS) that affect skin and / or mucosa

Classification of urticaria : 

M.Y.A.Mawla ZAGAZIG Classification of urticaria Urticaria : acute or chronic. Acute urticaria is defined as urticaria that has been present for less than 6 weeks. Chronic urticaria is defined as urticaria that has been continuously or intermittently present for at least 6 weeks. The 6-week period is a guide and not an absolute demarcation. When no underlying cause is found, chronic urticaria is referred to as chronic idiopathic urticaria (CIU).

Angieoedema : 

M.Y.A.Mawla ZAGAZIG Angieoedema Angioedema : involves swelling of the deepdermal and subcutaneous/submucosal tissues..

Pathophysiology of urticaria: : 

M.Y.A.Mawla ZAGAZIG Pathophysiology of urticaria: Skin lesions and pruritus : caused by an allergic or nonallergic mechanism. Histamine is an important biochemical mediator in urticaria causing the classic wheal-and-flare response observed with urticaria. Mast cells are the major histamine-releasing cells of the skin. The mast cell possesses high-affinity receptors for immunoglobulin E (IgE). In allergic reactions, adjacent IgE molecules, which are bound to the surface of mast cells by the high-affinity IgE receptors, are cross-linked by allergens, leading to the release of histamine and other mediators. Nonallergic mechanisms : (aspirin, neuropeptides NSAIDs, opiates, succinylcholine, , polymixin, ciprofloxacin, rifampin, vancomycin, some beta-lactams).They induce direct degranulation of mast cell

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Pathophysiology of urticaria : 

M.Y.A.Mawla ZAGAZIG Pathophysiology of urticaria Neuropeptides : involved in emotional exacerbation of urticaria. Basophils also possess the high-affinity IgE receptor and may be involved in urticaria. Other inflammatory cells are recruited into the lesional area in urticaria, particularly in chronic urticaria. These cells can release cytokines and chemokines that can cause histamine release or otherwise contribute to the pathology.

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Histopathology : 

M.Y.A.Mawla ZAGAZIG Histopathology A lymphocytic infiltrate is commonly found in the lesions of both acute and chronic types of urticaria. Autoimmune-mediated chronic urticaria lesions have a mixed cellular infiltrate ( lymphocytes, polymorphonuclear leukocytes (PMNs), and other inflammatory cells). Some patients have vasculitis on skin biopsy. A spectrum in histopathology seems to exist, ranging from lymphocytic to vasculitic, correlating with disease severity, from mild to severe.

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Mortality in urticaria : 

M.Y.A.Mawla ZAGAZIG Mortality in urticaria Mortality is rare, unless the condition is accompanied by severe anaphylaxis or severe respiratory tract angioedema.

Anaphylaxis : 

M.Y.A.Mawla ZAGAZIG Anaphylaxis Anaphylaxis: a systemic syndrome of immediate hypersensitivity caused by an IgE-mediated release of mediators from mast cells and basophils, presents clinically with bronchospasm, angioedema, hives, and cardiovascular collapse. Anti-IgE autoantibodies : as a possible cause of idiopathic anaphylaxis,. Such autoantibodies act on mast cells to crosslink the FcERI or IgE bound to this Fc receptor.

History taking in urticaria : 

M.Y.A.Mawla ZAGAZIG History taking in urticaria Typical features Typical lesions are described as edematous pink or red wheals of variable size and shape, with surrounding erythema. The lesions :generally pruritic. A painful or burning sensation may be described (such lesions are often associated with angioedema). Pruritus of nonlesional skin occurs. Dermographism:Itching, erythema, and a raised wheal occur in areas that are scratched or stroked . Individual lesions fade within 24 hours With delayed pressure urticaria, lesions last as long as 48 hours. The lesions of urticarial vasculitis, : palpable and purpuric, lasting for several days or more and leading to residual hyperpigmented changes.

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Questions asked to determine possible allergic vs non allergic causes : 

M.Y.A.Mawla ZAGAZIG Questions asked to determine possible allergic vs non allergic causes Are the hives associated with any foods? Have any new foods been added to the diet? Is the patient taking any regular medications or have any new medicines been started? aspirin, nonsteroidal anti-inflammatory drugs (NSAIDs), antibiotics, over-the-counter medications, herbs, and supplements. Does the patient have any recent or chronic infections? Are the hives caused by any physical stimuli (eg, heat, cold, pressure, vibration)? Does the patient have any chronic medical conditions?

Questions asked …………… : 

M.Y.A.Mawla ZAGAZIG Questions asked …………… Is the urticaria associated with any substances that are inhaled or in contact with the skin (which may occur in an occupational setting)? Is the urticaria associated with insect bites or stings?

Physical examination in urticaria: : 

M.Y.A.Mawla ZAGAZIG Physical examination in urticaria: Features of anaphylaxis (eg, hypotension, respiratory distress, stridor, gastrointestinal distress) Angioedema(deep tissue or submucosal edema). resembles idiopathic anaphylaxis, but other anaphylactic symptoms are absent Angioedema tends to progress slowly, often painful, and without pruritus. Look for typical skin lesions: edematous pink or red wheals of variable size and shape, with surrounding erythema.. Lesions that are purpuric, nonblanchable, palpable with residual pigmented changes are characteristic of urticarial vasculitis. Examine for dermographism.

Features of angioedema : 

M.Y.A.Mawla ZAGAZIG Features of angioedema Angioedema : to progress slowly, often painfully, and without pruritus. A positive family history of similar symptoms offers an obvious clue to the role of this disease in a patient's symptoms. Episodes : provoked by dental procedures or other causes of local trauma. Making the diagnosis is important because life-threatening symptoms of upper airway obstruction may not respond to epinephrine. The manifestations : prevented by daily doses of androgens such as danazol or stanozolol, which increase the ability of hepatic cells to make C1 esterase inhibitor. Diagnosis : decreased levels of C4, CH50, and C1 esterase inhibitor concentration or function. An acquired form : associated with lymphoproliferative and autoimmune diseases.

Causes of urticaria : 

M.Y.A.Mawla ZAGAZIG Causes of urticaria Food allergies:in acute urticaria and urticaria in children(eg. food additives or preservatives beverages, such as spoiled fish (scombroidosis), aged cheeses, or red wine) Drug allergies Antibiotics, such as penicillin, Urticarial reactions to penicillin lasting as long as 14 days after a course of treatment has stopped. Contact urticaria : an allergic reaction to a substance that comes into contact with the skin (eg, an occupational exposure). Papular urticaria : caused by insect bites; the lesions may last longer than 24 hours. Other immediate hypersensitivity allergic reaction to an ingested, inhaled, or percutaneously inoculated substance (eg, latex, stinging insects) Nonallergic release of mediators A number of drugs. hypersensitivity. Radiocontrast media sensitivity is not related to iodine, fish, or shellfish allergy.

Medical causes of urticaria : 

M.Y.A.Mawla ZAGAZIG Medical causes of urticaria Infectious causes A cute viral syndromes, hepatitis (A, B, and C), Epstein-Barr virus, and herpes simplex virus. Chronic parasitic infections. Sinusitis, cutaneous fungal infections, Helicobacter pylori infection. Cryoglobulinemias (eg, associated with hepatitis C, chronic lymphocytic leukemia) 3 -Serum sickness 4-Other immune complex–mediated inflammation

Physical causes (physical urticaria) : 

M.Y.A.Mawla ZAGAZIG Physical causes (physical urticaria) Cold Pressure: A delayed response to pressure (delayed pressure urticaria) occurs in up to 40% of patients with CIU. Vibration Cholinergic (triggered by heat, exercise, or emotional stress) Sunlight Water Dermographism . Exercise

Chronic idiopathic urticaria (CIU) : 

M.Y.A.Mawla ZAGAZIG Chronic idiopathic urticaria (CIU) Urticaria : no specific cause is identified by history, physical examination, or laboratory findings. Clinical features Daily, or almost daily, occurrence of urticarial wheals for at least 6 weeks. Angioedema occurs concurrently with CIU in about 50% of cases and delayed pressure urticaria in about 40%. The individual urticarial wheals last longer—at least 8 to 12 hours. Unlike UV wheals, wheals of CIU do not cause residual pigmentation. Systemic symptoms are minimal.

Lab Studies in a case of urticaria: : 

M.Y.A.Mawla ZAGAZIG Lab Studies in a case of urticaria: Skin tests or radioallergosorbent assay test (specific IgE) Common screening laboratory tests that are ordered are as follows: CBC with differential Total eosinophil count Sedimentation rate Urine analysis Liver function tests Evaluation of the complement system, including total hemolytic complement (CH50), C3, and C4 with prominent angioedema and rticarial lesions lasting more than 24 hours. Thyroid studies, including thyroid autoantibody levels (antimicrosomal, antithyroglobulin): Chemistry panel Stool analysis for ova and parasites H pylori workup Hepatitis B and C workup Sinus radiography (if symptomatic) Antinuclear antibody (ANA) Rheumatoid factor Cryoglobulin levels . Autologous serum skin testing: for CIU

Medical Care of cases of urticaria: : 

M.Y.A.Mawla ZAGAZIG Medical Care of cases of urticaria: Acute urticaria (<6 wk) : try to pinpoint a trigger In a small number of cases, a pattern may emerge, pinpointing the offending agent. Avoidance If a trigger can be identified Aspirin, NSAIDs, opiates, and alcohol :are avoided Intravenous gammaglobulin, plasmapheresis, and cyclosporin :n severe urticaria of the autoimmune type. Colchicine and dapsone : in urticarial vasculitis, because of their ability to modulate PMN function. Antileukotriene agents : provide a synergistic response when used in conjunction with antihistamines.

Adrenergic agents : 

M.Y.A.Mawla ZAGAZIG Adrenergic agents Epinephrine: Any patient who has had a potentially life-threatening allergic reaction should have injectable epinephrine available for use at all times (Any use of epinephrine necessitates an immediate evaluation in the nearest emergency department Dose: 0.2-0.5 mg IM/SC single dose; can be repeated in 15- to 20-min intervals IM administration has been associated with a faster time of onset than S.C. Paediatric Dose :0.01 mg/kg, up to 0.5 mg, IM/SC single dose; can be repeated in 15- to 20-min intervals prnIM administration has been associated with a faster time of onset than SC . Contraindications: Documented hypersensitivity; coronary insufficiency; cardiac arrhythmias; glaucoma se with caution during labor (may delay second stage of labor

THERAPY OF ANGIOEDEMA : 

M.Y.A.Mawla ZAGAZIG THERAPY OF ANGIOEDEMA Androgens -- Synthetic attenuated androgens (eg, danazol, stanozolol) taken prophylactically increase the serum concentration of C1INH, presumably by enhancing the function of the C1INH gene. When danazol is used prophylactically in adolescents or preadolescents, the concentration of C1INH and C4 are increased in the plasma. Danazol 200 mg/d PO initially; if abdominal discomfort recurs, increase to 400 mg/d PO for 1-2 mo; once symptoms are controlled, reduce dose to 200 mg/d PO; continue attempt to titrate downward to minimum effective dose .

THERAPY OF ANGIOEDEMA 2 : 

M.Y.A.Mawla ZAGAZIG THERAPY OF ANGIOEDEMA 2 Antifibrinolytic agents -- Used successfully as preventive therapy. Their effect may depend on physiologic or pathologic enhancement of plasminogen activation in blood, which may promote activation of C1INH Aminocaproic acid (4-5 g) IV over 1 h initially, followed by 1 g/h IV for 8 h; dilute IV solution to obtain concentration of 1 g/50 mLLength of treatment may be adjusted depending on response of patient C1-esterase inhibitor, human (investigational Prophylaxis: 500-1000 U IV for 2h prior to surgeryAcute treatment: 500-1000 U IVDose can be increased significantly depending on history and seriousness of previous attacks Complement replacement agents: Fresh frozen plasma (FFP) 2 U IV initially; may be gradually increased until improvement of symptoms observed

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M.Y.A.Mawla ZAGAZIG 180 Adult single dose for allergic rhinitis is 120 mg.

Non & Minimally Sedating Anti histamines in Urticaria : 

M.Y.A.Mawla ZAGAZIG Non & Minimally Sedating Anti histamines in Urticaria Cetirizine (Zyrtec):10mg :Minimally sedating Fexofenadine(Telefast):180mg Loratadine(Claritin):10mg Desloratadine(Clarinex):5mg

H2antagonists (antihistamines) : 

M.Y.A.Mawla ZAGAZIG H2antagonists (antihistamines) Cimetidine (Tagamet) -- If no response to H1 antagonist alone occurs, coadministration with this H2 antagonist can be useful to treat urticaria. Adult Dose: 300 mg PO qid or 400 mg PO bid Pediatric Dose: Infants: 10-20 mg/kg/d PO q6hChildren: 20-40 mg/kg/d PO in divided doses separated at least 6 h Interactions: Can increase blood levels of theophylline, warfarin, tricyclic antidepressants, triamterene, phenytoin, quinidine, propranolol, metronidazole, procainamide, and lidocaine

Corticosteroids in urticaria : 

M.Y.A.Mawla ZAGAZIG Corticosteroids in urticaria Prednisolone: Reduces capillary permeability. Adult Dose: 40-60 mg/d PO divided 1-2 doses per d Paediatric Dose: .5-2 mg/kg/d PO divided 2-4 doses per d Interactions: Decreases effects of toxoids (for immunizations). Phenytoin, carbamazepine, barbiturates, and rifampin decrease effects of corticosteroids

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Thank you : 

M.Y.A.Mawla ZAGAZIG Thank you Thank you

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