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ABIM Allergy Immunology Review 2014

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1 ABIM Allergy Immunology Review 2014
Richard D. deShazo, MD with thanks to the many colleagues from whom I have borrowed material

2 Questions: What are the major diagnostic criteria for asthma?
How can you separate asthma from COPD? What does a positive methacholine inhalation test mean?

3 Spirometry before and after an inhaled bronchodilator showing reversible airway obstruction

4 Asthma Diagnosis FEV1 improvement of 12% and 200mL after beta-agonist is evidence for asthma according to ATS Decreased FEV1/FVC ratio 20-39yr 85% 40-59yr 80% 60-80yr 70% Methacholine challenge – not diagnostic of asthma + with 20% decrease FEV1 with <8-16mg/mL True benefit is in negative predictive value Question: What does a positive methacholine inhalation test mean? Answer: Airways hyperreactivity of any cause; ie viral infections, allergic inflammation, etc. MKSAP Spirometry should be performed in patients with suspected asthma to confirm the presence of airway obstruction and reversibility. Measurement of peak expiratory flow rates can be used to monitor airway obstruction at home and to assess the relationship between symptoms and airway obstruction. The methacholine challenge test has a high negative predictive value for asthma, and a negative test can therefore be used to exclude asthma.

5 Question: What test should be considered in a young asthmatic female with recurrent episodes of wheezing poorly responsive to bronchodilators?

6 Vocal Cord Dysfunction Is Not Asthma, But May Occur In Asthmatics
Flow volume loops showing maximum inspiratory and expiratory flow-volume relationships in a patient with vocal cord dysfunction during asymptomatic (left) and symptomatic (right) periods. Note also the marked adduction of the vocal cords with severe reduction of the glottic aperture during a symptomatic period (right) of airway obstruction. Question: What test should be considered in a young asthmatic female with recurrent episodes of wheezing poorly responsive to bronchodilators? Answer: Flow-volume loop Question: Where in the respiratory cycle do abnormalities occur in vocal chord dysfunction syndrome? Answer: Inspiration MKSAP Exercise-induced asthma: Exercise-induced asthma is confirmed by exercise challenge testing (to greater than 85% of maximal predicted heart rate) with postexercise spirometry showing a 15% or greater fall in FEV1. Vocal cord dysfunction can be confirmed by flow volume loops when the patient is symptomatic, which show an inspiratory cut-off, or laryngoscopy, which shows abnormal adduction of the vocal cords. Cough-variant asthma: The diagnosis of cough-variant asthma is suggested by the presence of airway hyperresponsiveness and confirmed when cough resolves with a trial of inhaled albuterol.   

7 Vocal Cord Dysfunction
Female 2:1 Many (up to 1/3) can have coexist asthma Athletes Difficulty getting breath in Bronchodilator non responsive Differential Diagnosis of Asthma

8 2 Questions: What is the likely diagnosis in a man with persistent cough, shortness of breath, dyspnea, and episodic wheeze that developed after working as a police officer in the 9/11 exposure area in NYC? A 22 year old with recent onset cough and wheeze works in a custom sailboat shop in Biloxi sealing the inside of boats with epoxy resins. What is the most likely low molecular weight chemical causing his symptoms?

9 Reactive Airways Dysfunction Syndrome (RADS): Not Asthma
Treatment – Time + corticosteroids Occurs after a single inhalation of a caustic irritant in a non-asthmatic Obstruction on PFT (+) methalcholine inhalation challenge Limited PFT-response to beta-agonists Not “reactive airways disease” Question: What is the likely diagnosis in a man with persistent cough, shortness of breath, dyspnea and episodic wheeze that developed after working as a police officer in the 9/11 exposure area in NYC? Answer: RADs MKSAP Exposure to high levels of irritants (for example, chlorine gas, bleach, or ammonia) can result in significant airway injury, which can lead to persistent airway inflammation and dysfunction with airway hyperresponsiveness and obstruction. The reactive airways dysfunction syndrome follows a single, accidental inhalation of high levels of a nonspecific respiratory irritant in patients who typically do not have a history of asthma; the diagnosis is confirmed by a positive methacholine challenge test.

10 Occupational Asthma Basically, asthma occurring because of exposure at work. Testing difficult, so sometimes need to do pulmonary evaluation at the workplace. (Start with peak flows at work versus home). Earlier you diagnose the better, because may lead to permanent asthma even when removed from environment. Question: A 22 year old with recent onset cough and wheeze works in a custom sailboat shop in Biloxi sealing the inside of boats with epoxy resins. What is the most likely low molecular weight of chemical causing his symptoms? Answer: Trimellitic anhydride MKSAP Reviewing a patient’s occupational exposures, the job process and tasks, and substances involved in the patient’s activities is necessary for an evaluation for possible occupational asthma. Asthma syndromes: Occupational, RADS, cough-variant, asthma – GER, allergic bronchopulmonary aspergillosis, (ABPA), EIB, vocal chord dysfunction, aspirin sensitive

11 Risk Factors for Asthma Death
Previous life-threatening asthma such as respiratory arrest Hospitalization or ED visit for asthma within the last year Use of 2 or more canisters of rescue inhaler/month Poor perception of hypoxia or airway obstruction Psychosocial disturbance Question: A 22 year old chronic asthmatic presents with an exacerbation of symptoms. Blood gases show a pH of 7.40, p O2 of 100 and p CO2 of 43. What should be done for this patient? Answer: Admit to ICU MKSAP According to the newest National Asthma Education and Prevention Program’s guidelines, admission to the intensive care unit is recommended for symptomatic patients with even mild carbon dioxide retention (PCO2 greater than 42 mm Hg) or severely decreased lung function despite aggressive bronchodilator treatment (persistent FEV1 or peak expiratory flow less than 40% of predicted) Physical findings suggesting impending respiratory failure in asthma. Decreased breath sounds, accessory muscle use, sternocleidomastoid or suprasternal retractions, inability to speak in full sentences, and paradoxical pulse greater than 15 mm Hg are associated with severe airflow obstruction, although the absence of these findings does not necessarily exclude the presence of a high-risk exacerbation. The initial physical examination and findings are less predictive of the clinical course in a patient with asthma than the response to bronchodilators. Wheezing may become more prominent in the early stages of recovery owing to improved airflow through narrowed airways.

12 Classification of Asthma Severity
Components of Severity Mild Moderate Severe Impairment Intermittent ----Persistent---- Symptoms <2 days/weeks >2 days/week but not daily >1 x/week but not nightly Throughout the day Nighttime awakenings <2 x/month 3-4 x/month Often 7 x/week SABA use for symptom control (not prevention of EIB) < 2 days/week >2 days/week but not more than 1 x/d Daily Several times a day Interference with normal activity None Minor limitation Some limitation Extremely limited Lung function Normal FEV1 between exacerbations FEV1 >80% of predicted FEV1/FVC normal FEV1 >80% of predicted FEV1/FVC normal FEV1 >60% but <80% of predicted FEV1/FVC reduced 5% FEV1 <60% of predicted FEV1/FVC reduced >5% Risk Exacerbations (consider frequency and severity) 0-2/year >2/year Question: A 40 year old asthmatic reports that he awakens from sleep 3 times/ mo with cough and wheeze. What underlying reversible causes of asthma could contribute to this problem? What class of medication should be prescribed? Answer: Reflux, PPI; Anti-inflammatory asthma drug Most common asthma/sinusitis/nasal polyp syndromes: Aspirin sensitive asthma, allergic fungal sinusitis, cystic fibrosis, Churg-Strauss vasculitis

13 Asthma Pharmacotherapy
Rescue medicines Short acting agonists (SABA) Anticholinergic Controller medicines Inhaled corticosteroids (ICS) Long acting agonists (LABA) Leukotriene receptor antagonists (LTRA) Combinations of ICS & LABA Anti-IgE (omalizamab) Others Inhaled Asthma Drugs MKSAP The regular use of inhaled corticosteroids in patients with asthma improves lung function, decreases airway hyperresponsiveness, reduces asthma exacerbations, and reduces asthma-related mortality. The proper technique for using inhalers is necessary for an adequate therapeutic response in asthma; patients should be shown the proper technique, and any patients with poorly controlled disease should be evaluated for proper technique. The response to inhaled bronchodilators is more predictive of the clinical course in a patient with asthma than initial physical examination and findings. Perioral dermatitis is a papular and pustular eruption that appears around the mouth and is usually caused by the use of topical or inhaled corticosteroids. Allergy evaluation should be considered in patients with asthma who require more than minimal medication to control their disease. Advair ® = fluticasone (ICS) + salmeterol (LABA) Symbicort ® = budesonide (ICS) + formotorol (LABA) Combivent ®/DuoNeb ® = albuterol + ipratropium Pulmocort ® = budesonide (ICS) Foradil ® = formoterol Serevent ® = salmeterol Flovent ® = fluticasone

14 Stacking Asthma Drugs MKSAP
Inhaled corticosteroids are the cornerstone of therapy for persistent asthma. Adding long-acting β2-agonist inhalers in patients whose asthma is not controlled on low- to medium-dose inhaled corticosteroids is more effective than doubling the dose of inhaled corticosteroids. Because of significant drug-drug interaction, toxic serum concentrations of theophylline can be reached in a patient who previously had stable levels but who begins taking an interfering medication, such as a fluoroquinolone antibiotic. Step-up therapy for asthma: A short course of oral corticosteroids may help restore asthma control in previously well-controlled patients who have developed unstable disease as a result of a respiratory tract infection. Step-down therapy for asthma: Consider decreasing doses or discontinuing add-on drugs if symptoms are stable for 3 months. Leukotriene modifiers may be appropriate for patients with aspirin-sensitive asthma, exercise-induced asthma, and virus-induced wheezing. In patients with difficult-to-control asthma, lifestyle modifications and a trial of acid suppression with a proton pump inhibitor for potential gastroesophageal reflux disease should be considered.

15 What Meds Should You Think About Adding On In Severe Asthma?

16 Things to consider in the out of control asthmatic
Vocal cord dysfunction GERD Sinusitis Churg-Strauss syndrome ABPA Compliance/technique Question: A 25 year old with chronic allergic rhinitis has a URI. This leads to an acute asthma exacerbation which does not respond to step-up therapy. What should be done next? Answer: A sinus CT MKSAP Patients with allergic bronchopulmonary aspergillosis typically have eosinophilia, elevated serum levels of circulating IgE (total and specific IgE against Aspergillus fumigatus), and a positive skin test for Aspergillus. Poor inhaler technique is a major reason why patients with asthma do not respond well to specific asthma therapy. Gastroesophageal reflux disease complicating persistent asthma: In patients with asthma who have increased nocturnal symptoms despite adequate daytime control, a trial of gastric acid suppression therapy is warranted. In a patient taking high-dose inhaled corticosteroids as part of therapy for persistent asthma whose disease is stable, reducing the dose of corticosteroids should be considered to prevent therapy-related side effects. Long-term inhaled corticosteroids are safe and effective in treating asthma during pregnancy. Leukotriene inhibitors may be useful in patients with aspirin sensitive asthma, exercise induced asthma, and viral-induced wheezing. Avoid them in patients with psychiatric disorders.

17 How Do You Assess Asthma Control?

18 How Do You Use A Metered Dose Inhaler (MDI)?

19 What Does Every Asthmatic Have to Have?

20 3 Eosinophilic Lung Diseases
Churg-Strauss Vasculitis Sinusitis, asthma, eosinophilia, vasculitis Transient pulmonary infiltrates Small vessel vasculitis Neuropathies (mononeuritis multiplex) MPO- ab/P-ANCA test (+) in about 50% RX: steroids (cyclophosphamide/azathioprine in severe) ? relationship to LTRA Question: A 50 year old lawyer with chronic sinusitis and asthma presents with a left foot drop. What diagnostic test would be most definitive in establishing a diagnosis? Answer: Biopsy of affected nerve showing vasculitis of blood vessels supplying the nerve. MKSAP Anti-IgE antibody: Omalizumab reacts with the Fc portion of the IgE antibody and binds to free circulating IgE, thus preventing IgE attachment to basophils and mast cells. It is approved for moderate and severe asthma. Dose is adjusted on the basis of serum IgE levels and patient’s weight. It can cause anaphylaxis and costs up to $36,000 per year. Very few controlled trials to demonstrated efficacy of allergen immunotherapy (allergy shots) in asthma have been performed. Immunotherapy for house dust mite has been shown to be effective in asthma. It has been shown to be effective to many allergens in allergic rhinosinusitis. Theophilline levels are altered by many drug interactions and the drug has a narrow therapeutic window. Avoid it.

21 Allergic Bronchopulmonary Mycosis (ABPM)
Includes allergic bronchopulmonary aspergillosis (ABPA) and other allergic mycoses Criteria Asthma Fleeting pulmonary infiltrates High total IgE (>1000 ng/mL or 417 kU/L) Positive immediate skin test (IgE) to fungus (A. fumigatus, etc.) or Positive RAST test Elevated serum specific IgE to fungus (A. fumigatus, etc.) CT with central bronchiectasis reflects chronic disease Question: A 38 year old with chronic asthma reports coughing up plugs of mucous that look like gravel and bronchial casts. His total IgE level is 1200 IU and his total eosinophil count is 2200 cells/mm3. What would be the next step in diagnosing his problem? Answer: Allergy skin tests to fungi.

22 Treatment of ABPM Oral corticosteroids Follow total serum IgE and CXR
No long term studies on Itraconazole as steroid sparing agent

23 Hypereosinophilic Syndrome
No increase asthma but may have allergic – like dermatitis Paramalignant syndrome Peripheral blood eos count >1500 µL for at least 6 months Absence of any other known cause of eosinophilia Presumptive signs/symptoms of organ invasion by eosinophils 9:1 male:female Systemic disease Cardiac disease (thrombosis, fibrosis, necrosis) is the most common cause of death FIP1L1/PDGFA fusion-gene present in some patients HES Patients with HES have features common to myeloproliferative disorders including increased serum B12, splenomegaly, cytogenic abnormalities, myelofibrosis, anemia, and myeloid dysplasia. In some HES patients there is a defect in the gene for the platelet derived growth factor and receptor yielding the fusion gene FIP1L1/PDFGA detectable by flurosence in situ hybridization (FISH) or RT-PCR. These patients appear to have chronic eosinophilic leukemia. Serum tryptase levels are also usually elevated in the myeloproliferative HES varients.

24 Organs Involved in Hypereosinophlic Syndrome
Heart Skin, Lungs, GI tract, Nervous System Vascular thrombosis is a feared complication Treatment Steroids Hydroxyurea Interferon α Gleevac (imatinib) - for FIP1L1-PDGFRα gene rearrangement

25 Question: A 50 year old woman with asthma and atopic dermatitis has her 3rd episode of food impaction. What findings should be sought from endoscopy to confirm the diagnosis?

26 Eosinophilic Gastroenteritis
Atopic individuals Usually in upper tract Food impaction in esophagus Mucous furrowing and white specks on endoscopy More than 12 eosinophils/HPF on biopsy Food allergy in some Treated with non-absorbable steroids MKSAP Eosinophilic esophagitis is characterized by eosinophilic infiltration of the esophageal mucosa. Clinically, the disease most commonly presents with dysphagia or food impaction in atopic men in their third to fourth decades of life. For many years considered a pediatric disorder, the disease (or at least its recognition) appears to be rapidly increasing in adults. Diagnosis is suggested from clinical context, supported by endoscopic findings of mucosal furrowing or raised white specks (thought to represent eosinophilic microabscesses), and confirmed by histologic examination of the esophageal mucosa. Besides disimpaction of a food bolus, initial treatment is medical because of concerns about mucosal friability and esophageal perforation in the setting of dilatation. Swallowed topical corticosteroids (fluticasone propionate or beclomethasone) have been shown to produce clinical remission in up to 50% of adults. Proton pump inhibitors do not appear to be effective. Case reports citing the efficacy of leukotriene receptor antagonists have yet to be confirmed in randomized trials. Swallowed liquid corticosteroids are the first-line therapy for eosinophilic esophagitis

27 Question: A 50 year old bird breeder has persistent shortness of breath, cough, and dyspnea. What should be done?

28 Hypersensitivity Pneumonitis
Fever, chills, malaise, cough, dyspnea Acute, subacute, chronic A systemic illness Also known as “extrinsic allergic alveolitis” Non allergic, not IgE mediated, no eosinophilia, no asthma except pigeon breeder’s (bird fancier’s) lung T-cell mediated CXR/CT scan abnormal: ground glass/diffuse alveolar pattern PFT – restriction not obstruction except for pigeon breeders Chronic  pulmonary fibrosis MKSAP Hypersensitivity Pneumonitis: Antigen Source and Associated Disease

29 Approach to Allergic Rhinitis
Characterize chronicity looking for seasonal variation. Ask for family history, indoor triggers and nasal itching. Swollen nasal mucosa usually more blue that red. Treat If there is treatment failure, do allergy skin tests or RASTs to determine specific allergen and consider allergy shots. Allergy shots are very effective for allergic rhinitis and insect anaphylaxis. MKSAP Differential Diagnosis of Allergic Rhinitis

30 Frequent Board Rhinitis Scenarios
Allergic Rhinitis Nasal steroids – first line treatment Antihistamines and leukotriene receptor antagonists – second line (about equal efficacy as second line agents) To prevent symptoms on planned exposures, chromolyn or antihistamines work best Allergen immunotherapy by subcutaneous or sublingual techniques MKSAP Symptoms of episodic allergic rhinitis can be prevented by use of intranasal chromolyn sodium before exposure to the allergen. Frequent Board Rhinitis Scenarios Look for… Diagnose… Systemic illness with nasal ulceration or chronic sinusitis Wegener granulomatosis Young person, nasal polyposis, chronic sinusitis, malnourishment, infertility, and chronic or recurrent bronchitis Cystic fibrosis Nonseasonal rhinitis with negative skin tests Vasomotor rhinitis Refractory congestion after chronic use of topical nasal decongestants Rhinitis medicamentosa Nasal congestion in the last 6 or more weeks of pregnancy Pregnancy rhinitis

31 Mimics of Allergic Rhinitis
Vasomotor rhinitis Rhinitis medicamentosa Nasal manifestations of systemic disease: Diabetes mellitus – mucor mycosis (nasal eschar; black crust) Wegener’s granulomatosis (saddle nose) Midline granuloma (saddle nose) Relapsing polychondritis (saddle nose) Sarcoidosis (bloody crusts) Cystic fibrosis (nasal polyps) CSF leak – check beta 2 transferrin (very specific) MKSAP Chronic vasomotor (nonallergic) rhinitis should be considered in patients who develop perennial nasal congestion and rhinorrhea after 20 years of age, whose symptoms are exacerbated by irritant rather than allergic triggers, and who do not have a family history of allergies. The pathophysiology of vasomotor rhinitis is less clear than that of allergic rhinitis, but the chemical mediators causing symptoms are similar in both conditions. Symptoms of vasomotor rhinitis include nasal congestion and rhinorrhea and may develop after exposure to odors, humidity, temperature change, and alcohol. Sneezing and itching occur less often than in allergic rhinitis. Some experts consider the diagnosis one of exclusion. Results of skin tests and radioallergosorbent tests are normal in patients with vasomotor rhinitis and can be used to differentiate this condition from allergic rhinitis. Some patients have both allergic and nonallergic rhinitis. Chronic nonallergic rhinitis is less responsive to therapy than is allergic rhinitis. Topical intranasal corticosteroids, topical intranasal antihistamine, and topical ipratropium are the most consistently effective treatments. Rhinitis medicamentosa refers to the syndrome of rebound nasal congestion after discontinuing topical α-adrenergic decongestant sprays. Symptoms may occur after using these sprays for 5 or more days and resolve with prolonged discontinuation of these agents. However, this patient has not used nasal sprays. Rhinitis may be induced by other drugs, as well. Medications generally associated with drug-induced rhinitis include aspirin, NSAIDs, oral contraceptive agents, angiotensin-converting enzyme inhibitors, prazosin, methyldopa, β-blockers, and chlorpromazine. Patients with asthma and nasal polyps may experience hives due to aspirin sensitivity.

32 Rhinosinusitis Syndromes with Nasal Polyposis
Immunodeficiency Cystic fibrosis Aspirin – sensitive respiratory disease Allergic fungal sinusitis Anosmia is a big tip off for polyps CT of Allergic Fungal Sinusitis Hyperattenuation seen within allergic mucin filling sinuses in fungal sinusitis (ethmoid sinus) Missing globe Mucoperosteal thickening in both maxillary sinuses

33 Acute & Chronic Sinusitis
MKSAP Acute sinusitis Antibiotics are unlikely to be effective for most patients with suspected acute sinusitis. Sinus CTs are not recommended in acute sinusitis because of the high false (+) rate.  Most cases of acute sinusitis are due to a virus; only 0.5% to 2% are caused by bacteria. Signs and symptoms are not reliable for diagnostic purposes. A meta-analysis found that no symptoms or signs, including unilateral facial pain, pain in the teeth, pain on bending, or purulent nasal discharge, were precise enough to establish the diagnosis. In most patients, symptoms last up to 2 weeks and resolve without additional studies or administration of antibiotics. Even though most patients with suspected acute sinusitis do receive antibiotics, there is little evidence to support the effectiveness of this practice. A randomized trial found that the duration of symptoms did not differ between patients who did and did not receive antibiotics. A meta-analysis found that although patients with more severe symptoms had a longer period of illness, antibiotics did not decrease symptom severity or duration of infection. However, some guidelines still recommend administration of antibiotics. If these agents are used, they should be limited to patients with at least two of the following findings: symptoms lasting longer than 7 days, facial pain, and purulent nasal discharge. The data as a whole suggest that if antibiotics are to be used, amoxicillin or doxycycline are adequate first-line agents. Trimethoprim-sulfamethoxazole is acceptable for β-lactam–allergic adults. The patient described here has only facial pain and does not meet the criteria for antibiotic administration by most guidelines. Chronic sinusitis Think antibody deficiency, nasal polyps or Wegener’s granulomatosis. Ethmoid cell

34 Question: A 30 year old nurse has a severe chronic dry and cracking dermatitis of both hands. What should you do first?

35 Irritant vs. Allergic Contact Dermatitis
Patch testing can detect both irritant and allergic contact dermatitis, if the tests are read at appropriate times. Consider a patch test a TB skin test. Positive results are erythema and induration maximum at hours. Question: A 30 year old nurse has a severe chronic dry and cracking dermatitis of both hands. Should you do first? Answer: Take a history to distinguish irritant vs allergic contact dermatitis. This patient has chronic hand dermatitis, the differential diagnosis of which includes allergic contact dermatitis (ACD) and irritant contact dermatitis. Several features of this patient’s dermatitis suggest ACD, including the relatively recent onset of her condition (as opposed to lifelong), pruritus, and improvement when she is away from work. Epicutaneous patch testing is the appropriate test to evaluate for ACD and may help distinguish this from other types of chronic eczematous dermatitis, including irritant dermatitis and atopic dermatitis. This patient has a history of childhood eczema and, with her history of allergic rhinitis and family history of asthma, may also have atopic dermatitis. Hairdressers are exposed to several allergens that not infrequently cause occupational ACD. Accelerants and other chemicals can also cause allergies. Gloves may not protect a sensitized individual from reactions to all allergens, because some chemicals are able to penetrate natural rubber latex and synthetic rubber gloves. MKSAP Allergic contact dermatitis: Epicutaneous patch testing is the gold standard for diagnosis of allergic contact dermatitis in patients with persistent eczematous dermatitis.

36 Most Frequently Positive Patch -Test Results
MKSAP Most Frequently Positive Patch -Test Results in Allergic Contact Dermatitis Allergen Common Route of Patient Exposure Nickel sulfate Jewelry Neomycin Topical antibiotics Balsam of Peru (marker for fragrance allergy) Cologne or perfume Fragrance mix Most personal care products; products labeled “unscented” may contain masking fragrances Thimerosal (frequently positive, but rarely relevant) Eye-care products (eye drops, contact lens solutions); preservative in some vaccines Sodium gold thiosulfate Jewelry; dental work Quaternium-15 (formaldehyde-releasing preservative) Personal care products Formaldehyde Bacitracin Topical antibiotic; may cross-react with neomycin Cobalt chloride Blue paint; vitamin B12 (cyanocobalamin); may cross-react with other metals, including nickel

37 Question: A 16 year old male with asthma has a chronic pruritic dermatitis in the flexural areas of his elbows and knees. What treatment would be best for this problem?

38 Atopic Dermatitis (Eczema) (1)
Atopic dermatitis with involvement of a flexural surface. The frequently involved anticubital and popliteal fossae. Question: A 16 year old male with asthma has a chronic puritic dermatitis in the flexural areas of his elbows and knees. What treatment would be best for this problem? Answer: Medium strength topical steroids and a moisturizer (Aquaphor) +/- antistaphlococcal antibiotics

39 Atopic Dermatitis (2) Increased susceptibility to
Staph aureus, HSV (eczema herpeticum), vaccinia Differential diagnosis Allergic contact dermatitis Irritant contact dermatitis (gloves) Cutaneous T – cell lymphoma An adult presenting with eczematous dermatitis with an erythrodermatous appearance by skin biopsy with genetic studies Atopic dermatitis is associated with abnormalities in the filaggrin gene which encodes for filaggrin protein important in statium corneam moisturization MKSAP Patients with atopic dermatitis are more susceptible to disseminated cutaneous herpes virus infection (eczema herpeticum). Locally disseminated herpes simplex virus infection as a complication of atopic dermatitis

40 Treatment of Atopic Dermatitis
Skin hydration/moisturization (Aquaphor) Medium strength topical corticosteroids and combinations (equal parts 0.1% triamcinolone and Aquaphor) Oral antibiotics (Staph) Antihistamines (control itch) Topical calcineurin inhibitors (protopic/ elidel)

41 MKSAP Clinical Clues to the
Diagnosis of Selected Causes of Erythroderma Cause History Physical Examination Findings Laboratory Findings Atopic dermatitis Personal or family history of atopic dermatitis, allergic rhinitis, or asthma; preexisting skin lesions; severe pruritus Lichenification Eosinophilia; elevated serum IgE level Psoriasis Personal or family history of psoriasis; preexisting psoriatic skin lesions; history of corticosteroid withdrawal Nail pitting, oil droplet sign, onycholysis; psoriatic arthritis Skin biopsy may be diagnostic Cutaneous T-cell lymphoma/Sézary syndrome Slowly progressive; extreme, intractable pruritus Lymphadenopathy; painful, fissured keratoderma; alopecia; leonine facies Peripheral blood smear with >20% Sézary cells; clonal T-cell population in skin, blood, and/or lymph node Hypersensitivity drug eruption Onset 3-6 weeks after start of a new medication; most common causative medications are allopurinol, aromatic anticonvulsants, dapsone, NSAIDs, sulfonamides, lamotrigine Facial edema; cervical lymphadenopathy; fine scale around nose; hepatosplenomegaly Leukocytosis with eosinophilia or atypical lymphocytosis; elevated serum aminotransferases; elevated blood urea nitrogen or serum creatinine levels Question: A 62 year old man presents with an erythematous eczematoid dermatitis which is poorly responsive to corticosteroids. What should be done next? Answer: a skin biopsy with special studies for cell clonality Erythroderma: A skin biopsy is always required in the evaluation of a patient with erythroderma.

42 Urticaria Classification of Urticaria Acute urticaria (<6 weeks)
Chronic urticaria (>6 weeks) Physical urticaria (pressure, cold, vibratory, etc.) Urticarial vasculitis Contact urticaria (touching a cat) Urticaria and angioedema as components of anaphylaxis Select epinephrine, antihistamines, and corticosteroids for acute episodes of angioedema (not HAE) with airway compromise or hypotension. Epinephrine is effective in racemic nebulized or by SC injection. Use antihistamines or corticosteroids in cases without airway compromise or hemodynamic instability.

43 Classification of Physical Urticarias
Characteristics Papular urticaria Associated with insect bites Cholinergic urticaria Occurs in response to an increase in core body temperature; lesions present as small papules with a prominent surrounding erythematous flare Delayed pressure-induced urticaria Patients often experience systemic symptoms Exercise-induced urticaria On a continuum with exercise-induced anaphylaxis; many patients only develop symptoms if primed by an ingested allergen prior to exercise Solar urticaria Some patients who appear to have solar urticaria prove to have lupus erythematosus or porphyria Cold urticaria Acquired cold urticaria is often related to an underlying infection, whereas familial cold urticaria represents an autoinflammatory syndrome Vibrational urticaria MKSAP Chronic idiopathic urticaria should not be treated with oral or topical steroids. A workup for hereditary angioedema should not be done on a patient with urticaria. Patients with HAE do not have hives.

44 Palpable purpura is a clinical sign of a small vessel (leukocytoclastic) vasculitis which may present as chronic urticaria.

45 Question: A 68 year old female with chronic mylogenous leukemia presents with non itchy swelling of the lips and throat. What diagnostic tests would be most useful to diagnose acquired angioedema?

46 Differential Diagnosis: Cutaneous and/or Laryngeal Swelling
Allergic reactions and anaphylaxis Idiopathic angioedema Drug induced angioedema Allergic contact dermatitis Autoimmune conditions Thyroid disorders Superior vena cava syndrome and tumors Cheilitis granulomatosa (Miescher’s cheilitis) and Melkersson-Rosenthal syndrome Trichinosis Low C-4 levels

47 Hereditary Angioedema
Autosomal Dominant Hiveless, itchless edema Hereditary angioedema form more likely to have symptoms precipitated by trauma - dental visit, surgery, auto accident, menses, puberty Visceral attacks may present as an acute abdomen with normal findings at surgery MKSAP Question: A child who has episodic abdominal pain develops genital edema when riding a bicycle. What is the diagnosis? Answer: Hereditary angioedema Obtain C2, C4, and C1 esterase inhibitor levels, usually decreased, and C1 esterace function (down in form with normal levels) to rule out HAE types 1 and 2. C2 levels are low in acute HAE and C4 levels are low in both acute episodes and remission. C4 and C1 esterase inhibitor levels are normal in food allergy, drug-induced angioedema (ACE inhibitors), type 3 angioedema (hormone sensitive angioedema), or idiopathic angioedema. There is an estrogen sensitive form (Type 3 HAE) which is autosomal dominant with normal C1 INH and C4 levels and results from a mutation causing increase kallikrein production. IV C1 esterase inhibitor concentrate and kallikrein inhibitors can be used for acute episodes of HAE. For long-term management of HAE, select danazol and stanozolol to elevate hepatic synthesis of C1 esterase inhibitor protein. Don’t be tricked: Epinephrine is not effective for HAE.

48 Symptoms: Painless, itchless angioedma with/without family history
Diagnosis of HAE5 HAE 1 - low C41, 2 low C1 INH, low C1INH-F HAE2 - low C41, 2 normal C1 INH, low C1INH-F Acquired – low C41, 3 low C1 INH, low C1INH-F, low Clq HAE3 – normal C4 4 normal C1 INH, normal C1INH-F, normal C1f level Always low during attacks SERPING1 gene abnormalities Antibodies to C1INH Abnormalities in Factor XII genes Family members should be screened in all types

49 Acquired Angioedema Associated with lymphoproliferative disorders
Have low C1q levels Mechanism unclear Low C1 of levels appear to reflect autoactivation Anti – C1 esterase inhibitor antibodies have been described as well

50 Tests for Hereditary/Acquired Angioedema
C4 – is a great screening test, but is normal in HAE Type 3 Test for C1 esterase inhibitor level and function to discriminate between two hereditary types Test C1q level for the acquired form Genetic testing for HAE Type 3

51 Therapy of HAE On-demand treatment
Treatment of attacks with upper airways symptoms is mandatory Acute attacks should be treated with C1INH (plasma derived) Berinert Escallantide (inhibits HMW Kininogen to bradykinen) Kalbitor Icatibant (bradykinen recepptor antagonist) Firazyr Intubation or trachestomy should be early in progressive airway obstruction Antifibronolytics are not to be used Procedural prophylaxis with procedures involving the upper airway is recommended without evidence Pregnancy – C1INH advised Long-term Prophylaxis C1INH - immunizations, screens Androgens – frequent screens for liver toxicity Antifibrolytics not recommended

52 First-line therapies for acute attacks of HAE include
Purified (C1INHRP) or recombinant (rhC1INH) human C1 inhibitor (various products available worldwide) Ecallantide, a kallikrein inhibitor (available only in the United States) Icatibant, a bradykinin B2 receptor antagonist (available in the United States and the European Union)

53 Question 23: What should be prescribed for a patient after treatment for acute anaphylaxis and how should it be used?

54 Anaphylaxis Usually starts with urticaria and itching but can present with syncope, hypotension, or erythoderma. Most common causes are foods (peanut/ tree nut ingestion, etc.), insect stings, drug allergy (beta lactam) Don’t forget latex, especially in medical spaces No obvious trigger- think mastocytosis (check serum mast cell tryptase) or idiopathic anaphylaxis All who have insect anaphylaxis patients require evaluation for venom immunotherapy MKSAP Urticaria Photo

55 Anaphylaxis Early Management
Hypotension - supine, IV (NS) Respiratory distress - oxygen and albuterol, intubation Epinephrine 1: cc IM for adults (note this is NOT the 1:10,000 dilution - (1mg/10ml) on crash carts Can repeat in 5 minutes If on beta blocker and not responsive to epi, consider glucagon 1mg IM, IV, SC Can start epi infusion if not responsive to IM epi (1:10,000) 1-3 mg over 3 min, then 3-5mg over 3 minutes, then 4-10ug/min infusion All who recover must leave with an Epipen®. Consider 1 mg of glucagon IV if on beta blocker Glucagon: Most people vomit when given glucagon injections. Prepare for that.

56 Cutaneous Mastocytosis
Have urticaria pigmentosa – reddish brown or tan macules. Darier’s sign Indolent, benign course Itching, burning skin, flushing, CNS symptoms

57 Criteria for Systemic Mastocytosis
Major Multifocal dense infiltrates of mast cells in bone marrow or extracutaneous organ Minor >25% mast cells are spindle-shaped or atypical Presence of c-kit point mutation Mast cells co-express Kit and CD2 Persistent serum tryptase >20ng/mL Need one major and one minor, or 3 minor Other: Skin involvement improves prognosis Insect stings may induce anaphylactoid reactions in patients with mastocytosis. Patients with insect venom anaphylaxis and idiopathic anaphylaxis need a resting mast cell tryptase to exclude.

58 Drug Reactions MKSAP Common Drug Reaction Patterns MKSAP
Selected Newly Described Cutaneous Drug Reactions

59 Vancomycin Reactions “Red man syndrome” – pruritis and erythema of face, neck, upper torso, occasionally with hypotension Non-immunologic release of histamine Not IgE mediated Rx: slow the infusion and pre-treat MKSAP The “red man syndrome” (RMS) is the most common adverse reaction to vancomycin. It is characterized by flushing, erythema, and pruritus involving primarily the upper body, neck, and face. It may be associated with back and chest pain, dyspnea, and hypotension. Calcium channel blockers may cause eczematous drug eruptions.

60 Anticonvulsant Hypersensitivity
Cause the “hypersensitivity syndrome” Deficiency of epoxide hydrolase Fever, maculopapular rash, generalized lymphadenopathy Node bx resembles Hodgkin’s Phenytoin, carbamazepine, phenobarb Can also cause DRESS: Drug reaction with eosinophilia and systemic symptoms. Rash, fever, multi-organ failure ½ actually have eosinophilia Acute facial edema in a patient with anticonvulsant-induced drug reaction with eosinophilia and systemic symptoms (DRESS).

61 Serum Sickness MKSAP: Drug reactions are most commonly caused by a few drugs: antibiotics (particularly penicillins, β lactams, and sulfonamides), anticonvulsants, NSAIDs, thiazide diuretics, and allopurinol. Clinical patterns associated with severe drug reactions include confluent erythema, skin pain, facial edema, fever, lymphadenopathy, mucosal erosions, widespread blistering, purpura, necrosis, dyspnea, hypotension, elevated serum aminotransferase levels, lymphocytosis, and/or eosinophilia.

62 Question 24: A 21 year old college student has recurrent sinupulmonary infections. What test in addition to HIV should be performed to exclude immunodeficiency?

63 Immunodeficiency Syndromes
T- cell (cellular immunity) – virus, fungi, protozoa, mycobacteria and other intracellular organisms Humoral (antibody mediated immunity) – infection with extracellular pyogenic organisms Haemophilus Pneumococcus Streptococcus Increased infections Recurrent respiratory infections Multiple systems involved Unusual organisms Malabsorption Big LNs or absent LNs Immunodeficiency Syndromes Presenting Pattern Defect Test Invasive skin infections Granulocyte (chronic granulomatous disease) Nitroblue tetrazolium dye test Viral, intravellular pathogenic, or fungal infections Cell-mediated Skin test response to PPD, mumps, and Candida antigen Sinopulmonary infections, bacteremia with encapsulated organisms Immunoglobulins Quantitative serum immunoglobulins and response to tetanus and pneumococcal vaccines Neisseria meningitidis meningitis and disseminated gonorrhea Terminal complement components (C5, C6, C6, C8, and C9) CH50 assay

64 Common Variable Immunodeficiency
Decreased IgG, M, A, normal to increased IgE Recurrent sinopulmonary infections Lymph tissue present or enlarged High incidence of autoimmune disease (22%) Increased risk of adenocarcinoma and lymphomas In addition to encapsulated organisms Giardia, yersinia, H. pylori, and H. jejuni are common MKSAP Common Variable Immunodeficiency Common variable immunodeficiency (CVI) is the most common primary immunodeficiency that presents during adult life. It is characterized by: Hypogammaglobulinemia and recurrent bacterial upper and lower respiratory infections. Recurrent infection with encapsulated bacteria (pneumococus, Haemphilus, staphylococcus) Giardia lamblia infection Chronic diarrhea (50% of patients) Malabsorption Some other considerations: Autoimmune manifestations are found in one-third of cases (hemolytic anemia, systemic lupus erythematosus, arthritis). CVI increases risk for gastric adenocarcinoma, intestinal lymphoma, and non-Hodgkin lymphoma. Most patients with isolated IgA deficiency are clinically normal, but symptomatic patients may present with recurrent respiratory infections and giardiasis. Select measurement of serum IgM, IgA, IgG (all low) and IgG subclasses (variably low) and ability to mount an antibody response to tetanus toxoid (protein) and pneumococcal vaccine (polysaccharide) antigens. Therapy – select IV immune globulin as first-line therapy for CVI Don’t be tricked – IV immune globulin is contraindicated in isolated IgA deficiency because these patients may have IgG or IgE antibodies directed against IgA. Blood transfusion is also contraindicated unless washed erythrocytes are used.

65 IgA deficiency Most common form of primary immunodeficiency (1:333)
IgA <5mg/dL (VERY LOW) Most patients with IgA def. are NORMAL Have increased risk of infections: collagen vascular , allergic, and GI disease, and malignancy Can make anti-IgA antibodies Leads to anaphylaxis with IgA containing blood products Question: How should a patient be evaluated for functional and quantitative antibody deficiency? Answer: Quantitative Immunoglobulins (IgG, IgM, IgA), Immunoglobulin sub-classes (IgG, IgG2 , IgG 3, IgG4), and serum antibody responses before and after initial or booster immunization to pneumococcal tetanus and other vaccines to see a 4 fold rise in titer.

66 IVIG Indicated for common variable immunodeficiency and specific antibody deficiency Not indicated for IgA deficiency Side effects Never transmitted of HIV Fever, chills, HA, muscle pain. Aseptic meningitis Renal failure (was due to osmotic load, not as common now), stroke, MI

67 Complement Deficiency
C2 deficiency (most common) – sepsis, pneumonia, meningitis, pyogenic arthritis with Strep pneumo C2 deficiency – increased risk of rheumatoid arthritis, SLE Terminal Complement Components – Neisseria sp infections Think about if recurrent meningitis or if unusual strain

68 Live Vaccines to Avoid in Cellular Immune Deficiency
Measles Mumps Oral Polio Rubella Varicella MMR Smallpox (vaccinia) Flumist Question: What vaccines should be avoided in patients with anaphylaxis to egg? Answer: Flu and yellow fever

69 Egg Allergy and Vaccines
Influenza and Yellow fever

70 Question 28: What dietary pattern do bedbugs follow?

71 “Breakfast, lunch, and dinner”
Bedbug Bites Often Occur in a Series “Breakfast, lunch, and dinner” Lesions are usually painless and appear as pruritic, urticarial-like papules. Dinner Question: Where in the bed to bedbugs hang out? Answer: Along the rib cords of mattresses. Lunch Breakfast

72 Bedbugs are nocturnal, hiding in cracks and crevices within 10 feet of a bed and especially on the cords of the mattress during the day and biting at night. Question: What infectious diseases have bedbugs been shown to transmit? Answer: None Fed Hungry Rib cords Blood spot Poop Rib cords

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