chronicurticariasinnwell...¡Delayed pressure urticaria/angioedema (DPUA) Swelling occurs 4-6 hours after exposure to pressure Carrying heavy bag, sitting on hard surface, standing

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Elise Sinnwell PA-C

CHRONIC URTICARIA: APPROACH TO EVALUATION AND MANAGEMENT

¡  None

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DISCLOSURES

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¡  Define the differences between acute and chronic urticaria including possible causes

¡  Describe typical clinical manifestations of urticaria and identify features that are concerning for urticarial vasculitis

¡  Describe common triggers for chronic urticaria including identifying patients with physical urticaria syndromes

¡  Identify when targeted lab evaluation or referral for allergy testing is indicated

¡  Describe appropriate stepwise management of chronic urticaria

LEARNING OBJECTIVES

1.  Is it urticaria/angioedema?

2.  Is it acute or chronic?

3.  Consider possible triggers including whether may have physical urticaria

4.  Consider dif ferential diagnosis 5.  Consider targeted lab evaluation 6.  Management

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OUTLINE

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IS IT URTICARIA/ANGIOEDEMA?

¡ Urticaria § Red/pink, raised, pruritic, wheal(s) of variable

shapes (round, oval, serpiginous) § May have central clearing § Blanch with pressure § Individual lesions last <24-48 hours and leave no

residual changes to skin Bernstein JA et al. The diagnosis and management of acute and chronic urticaria: 2014 update. J Allergy Clin Immunol.2014. 6


¡ Angioedema § Swelling of face, tongue, throat, genitalia, extremities

§ Non-pruritic, non-pitting edema § Approximately 40% of patients with urticaria have

accompanying angioedema

§ Angioedema w/o urticaria should prompt considerations of ACEI-induced, hereditary or acquired angioedema

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Bernstein JA et al. The diagnosis and management of acute and chronic urticaria: 2014 update. J Allergy Clin Immunol.2014. 8


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OUTLINE

¡ Duration<6 weeks ¡ May have identifiable

cause ¡ May be caused by IgE-

mediated response to allergen

Chronic

¡ Duration>6 weeks ¡ Cause is not identified

in majority of cases ¡ Not caused by IgE-

mediated response to allergen (with rare exception)

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IS IT ACUTE VS CHRONIC?

Acute

Bernstein JA et al. The diagnosis and management of acute and chronic urticaria: 2014 update. J Allergy Clin Immunol.2014.

¡ May be caused by §  IgE-mediated reactions (food,

hymenoptera, allergen IT) § Medications §  Infection

¡ Testing as indicated by history

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ACUTE URTICARIA


¡  Cause or associated systemic disorder cannot be identified in majority of cases

¡  Unclear pathogenesis for chronic urticaria

§  Autoimmune theory ¡  Reported with a variety of systemic disorders including

autoimmune disorders, thyroid disorders, malignancy, infection

¡  History and exam findings may dictate whether further

evaluation is needed but extensive work up is not recommended in majority of cases

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CHRONIC URTICARIA


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OUTLINE

¡ NSAIDS/ASA ¡ Heat/tight clothing ¡ Stress

¡ Alcohol

¡ Opiates

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TRIGGERS


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PHYSICAL URTICARIA

¡ Dermatographism ¡ Cholinergic urticaria ¡ Delayed pressure urticaria ¡ Cold urticaria ¡ Solar urticaria ¡ Vibratory urticaria ¡ Aquagenic urticaria

¡ Dermatographism § Most common type of

physical urticaria

§ Simple vs symptomatic § Test: Stroke skin with

firm object (tongue blade)

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PHYSICAL URTICARIA

Bernstein JA et al. The diagnosis and management of acute and chronic urticaria: 2014 update. J Allergy Clin Immunol.2014. Abajian M et al. Physical Urticarias and Cholinergic Urticaria. Immunol Allergy Clin N Am. 2014; 34: 73-88.

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¡ Cholinergic urticaria § “Pinpoint” (1-3 mm)

§ Exercise, hot water, strong emotions, sweating

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PHYSICAL URTICARIA


¡ Cholinergic urticaria

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PHYSICAL URTICARIA

¡ Delayed pressure urticaria/angioedema (DPUA) § Swelling occurs 4-6 hours after exposure to pressure

§ Carrying heavy bag, sitting on hard surface, standing on feet for long periods

§ Hands, feet, buttock

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PHYSICAL URTICARIA


¡ Cold urticaria: § Urticaria w/ exposure of skin to cold § Triggers: holding cold drink, lip/pharyngeal

symptoms w/ cold beverage § May trigger systemic reaction (swimming pool)

¡ Solar urticaria § Urticaria develops within 1-3 minutes of sun

exposure to skin

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PHYSICAL URTICARIA


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¡ Vibratory urticaria/angioedema: § Swelling and pruritus w/in 1-3 minutes of exposure to

vibratory stimulus § Triggers: working with machinery, riding motorcycle

¡ Aquagenic urticaria: § Urticaria develops w/in 30 minutes of skin contact w/

water § Hives are generally 1-3 mm in size

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PHYSICAL URTICARIA





4.  Consider dif ferential diagnosis

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OUTLINE

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Differential diagnosis Features

Cryoglobulinemia Cold induced lesions, purpura, Raynaud’s phenomenon, occurring on extremities

Urticarial vasculitis Lesions lasting >24-48 hours, hyperpigmentation See next slide

Mast cell disorders Urticaria pigmentosa. Episodic flushing, itching, hypotension, GI symptoms

Hypereosinophilic syndrome Total eosinophil count >1500/microL

Schnitzler syndrome Monoclonal gammopathy, fever, non-pruritic urticaria, bone pain

Cryopyrin-mediated periodic syndromes

Familial cold autoinflammtory syndrome Muckle-Wells syndrome

Pruritic urticarial papules and plaques of pregnancy

Urticarial plaques on abdominal striae in third trimester

Bernstein JA et al. The diagnosis and management of acute and chronic urticaria: 2014 update. J Allergy Clin Immunol.2014. Saini SS. Chronic Spontaneous Urticaria: Etiology and Pathogenesis. Immunol Allergy Clin N Am. 2014;; 34: 33-52.

¡  Suspect when lesions last >24-48 hours, leave residual ecchymosis or hyperpigmentation, painful lesions

¡  Requires skin biopsy for diagnosis ¡ May be isolated to the skin or may have systemic involvement

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URTICARIAL VASCULITIS

Bernstein JA et al. The diagnosis and management of acute and chronic urticaria: 2014 update. J Allergy Clin Immunol.2014. Venzor J et al. Urticarial Vasculitis. Clinical Reviews in Allergy and Immunology. 2002; 23: 201-216.

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1.  Is it urticaria/angioedema? 2.  Is it acute or chronic?


4.  Consider dif ferential diagnosis 5.  Consider targeted lab evaluation

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OUTLINE

¡ Majority of cases of chronic urticaria is not associated with systemic disorder or external cause

¡  Evaluation should be limited and targeted based on history, physical exam, ROS

¡  Appropriate limited testing might include: §  TSH §  ESR/CRP §  CBC with differential §  LFTs

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TARGETED EVALUATION


27 Bernstein JA et al. The diagnosis and management of acute and chronic urticaria: 2014 update. J Allergy Clin Immunol.2014.




4.  Consider dif ferential diagnosis 5.  Consider targeted lab evaluation

6.  Management

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OUTLINE

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¡  Avoidance of triggers ¡  Education ¡ Medication management

§  Stepwise approach §  Alternative therapies

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MANAGEMENT

Bernstein JA et al. The diagnosis and management of acute and chronic urticaria: 2014 update. J Allergy Clin Immunol.2014. 30 Bernstein JA et al. The diagnosis and management of acute and chronic urticaria: 2014 update. J Allergy Clin Immunol.2014.

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H1 SECOND GENERATION ANTIHISTAMINES

¡  Example: loratadine, fexofenadine, cetirizine ¡ May begin at once or twice daily dosing ¡  Can be increased to 4 times standard dose (i.e., cetirizine 20

mg BID)

Bernstein JA et al. The diagnosis and management of acute and chronic urticaria: 2014 update. J Allergy Clin Immunol.2014. Staevska M, et al. The effectiveness of levocetirizine and desloratadine in up to 4 times conventional doses in difficult-to- treat urticaria. J Allergy Clin Immunol. 2010;125: 676-82.

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H1 FIRST GENERATION ANTIHISTAMINES

¡  Examples: diphenhydramine, hydroxyzine ¡  Side effects: drowsiness, anticholinergic side effects ¡  Can be tried initially with daily nighttime dosing


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H2 ANTAGONISTS

¡  Examples: ranitidine, cimetidine, famotidine ¡  Consider trial during step 2 therapy


LEUKOTRIENE RECEPTOR ANTAGONISTS

¡  Examples: montelukast, zafirlukast ¡  Consider trial during step 2 therapy

De Silva NL et al. Leukotriene receptor antagonists for chronic urticaria: a systemic review. Allergy, Asthma & Clinical Immunology. 2014; 10 (24): 1-6. Bernstein JA et al. The diagnosis and management of acute and chronic urticaria: 2014 update. J Allergy Clin Immunol.2014.

¡  Dapsone ¡  Sulfasalazine ¡  Hydroxychloroquine ¡  Cyclosporine ¡  Omalizumab (anti-IgE)

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ALTERNATIVE THERAPIES

Bernstein JA et al. The diagnosis and management of acute and chronic urticaria: 2014 update. J Allergy Clin Immunol.2014. 36 Bernstein JA et al. The diagnosis and management of acute and chronic urticaria: 2014 update. J Allergy Clin Immunol.2014.

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¡  Short term use may be considered ¡  Consider side effects (weight gain, hyperglycemia,

osteoporosis, adrenal suppression, gastritis, immunosuppression, etc)

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STEROIDS?


Step up Step down


¡  Urticaria typically presents with: §  red, raised wheal(s) of variable sizes and shapes, individual lesions

last <24-48 hours and leave no residual skin changes

¡  Acute urticaria:

§  has duration <6 weeks and may have identifiable cause

¡  Chronic urticaria: §  has duration >6 weeks and cause or associated systemic disorder is

not identified in majority of cases

¡  Some patients with chronic urticaria have nonspecific triggers: §  NSAIDS, tight clothing/heat, alcohol, opiates, stress

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TAKE HOME POINTS

¡  Physical urticaria: §  Is a subgroup of chronic urticaria in which primary trigger for

urticaria/angioedema is environmental/external stimulus

¡  Evaluation of patient with chronic urticaria:

§  Should be limited §  If any testing is done, should be targeted based on history and exam

¡ Management of patient with chronic urticaria: §  Includes avoidance, education/reassurance and medication

management

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TAKE HOME POINTS

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¡ 25 yo F presents with 3 month hx of hives that occur daily

¡ Describes as “very itchy spots

that come and go” ¡ Eyelid will swell shut sometimes ¡ Concerned about allergy and

has restricted her diet and avoided going outdoors

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CASE

¡ Hives seem to worsen after she takes ibuprofen

¡ Exam is notable only for

+dermatographism ¡ ROS completely negative ¡ Recent normal TSH,

ESR/CRP, CBC with differential, LFTs

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CASE

¡ Education/reassurance ¡ Recommend avoidance of

NSAIDs ¡ Start cetirizine 10 mg BID

¡ Follow up in 4 weeks

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CASE

Bernstein et al. The diagnosis and management of acute and chronic urticaria: 2014 update. J Allergy Clin Immunol.2014.

¡ At follow up, hives improved but still having some during day and very itchy at night

¡  Increase cetirizine to 20 mg BID and add hydroxyzine 25 mg QHS

¡ Return visit in 4-6 weeks ¡ Hives much improved, elect to

continue regimen for next 3 months

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CASE

Bernstein et al. The diagnosis and management of acute and chronic urticaria: 2014 update. J Allergy Clin Immunol.2014.

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Questions?

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chronicurticariasinnwell...¡Delayed pressure urticaria/angioedema (DPUA) Swelling occurs 4-6 hours after exposure to pressure Carrying heavy bag, sitting on hard surface, standing