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December 23, 2003

By Ernest N. Charlesworth, M.D.
Department of Allergy and Dermatology
Shannon Clinic, San Angelo, Texas
Clinical Associate Professor of Medicine, 
University of Texas Medical School at Houston

With a background in both dermatology and allergy, I have become well aware of the frustrations that urticaria presents to both the physician and the patient. Fifteen percent of the general population will experience urticaria at one time or another, and some of these patients will develop chronic urticaria. The latter frequently go from one physician to another in hopes of finding the elusive cause for their “hives.”

Today we know a little more about chronic idiopathic urticaria. In fact, we now recognize that 30 percent to 40 percent of patients with CIU have an autoantibody directed against the high affinity IgE receptor on mast cells and basophils. When the serums from these patients are analyzed, the following distinct subtypes can be identified:

1. Immunoreactive histamine releasing anti-Fc epsilon R1 autoantibodies in 26 percent
2. Immunoreactive anti-Fc epsilon R1 atuoantibodies without histamine release (15 percent)
3. Anti-IgE antibodies
4. Mast cell histamine releasing factor (9 percent)
5. Serum with no factor (41 percent)

The propensity to form antibodies has been extended to auto-thyroid antibodies in a subgroup of patients. Additiononally, it is recognized that patients with systemic lupus erythematosus may have urticaria. Urticarial vasculitis is another forme fruste of urticaria, which is a true leukocytoclastic vasculitis.

With the above brief introduction, there are a few questions that I would like to briefly address while remembering that there is nothing simple about urticaria. In fact, it might be best to think of urticaria as symptom and not as a disease. Just as there are many causes for a fever, there are many causes for urticaria. In reality, there is no such thing as idiopathic urticaria since there must be an underlying mechanism. A patient once asked me what idiopathic meant, and I answered that it “means that the doctor is an idiot.” In your practice, you will also find treating and evaluating urticaria as a humbling experience and at times you may feel that you are that idiot in idiopathic.

How should we evaluate a patient who has had an individual urticarial lesion for greater than 24 hours?
Now that is a good question, and it illustrates the importance of tying the clinical setting of the urticaria with mechanism of the disease. Briefly, urticaria, which remains in the exact same location, is urticarial vasculitis until proven otherwise. This is a histopathologic diagnosis, and it mandates the performance of a skin biopsy. The biopsy will show neutrophils invading the post capillary venules of the skin, associated with fibrin deposition, swelling of the intimal lining of the vessel and “nuclear dust.” Sometimes, one may see a polymorphic infiltrate with urticaria in which the lesions last 12-24 hours in the absence of an associated vasculitis. Bottom line: biopsy the skin.

In general, what are the essential features of physical urticarias that distinguish them from IgE mediated urticaria?
Simple question, complicated answer. The physical urticarias are a group of diseases in which symptoms develop simply by physical environmental changes. Mechanical sources produce dermatographism, delayed pressure urticaria and vibratory urticaria. Thermal changes are responsible for cholinergic and cold urticaria. Exposure to light, water and exercise are the trigger for solar urticaria, aquagenic urticaria and exercise-induced anaphylaxis, respectively. Physical agents are involved in 10 percent to 20 percent of all chronic urticarial reactions.

What recent advances in chronic urticaria should we review when preparing for our boards, and at what point should we refer a patient for a skin biopsy?
Well, the answer to the first part of the question is addressed in my introduction, above. For the boards, you should know the recent data regarding autoantibodies and the clinical subtypes that I discussed. In regard to knowing when to refer a patient for a skin biopsy, my answer is that you should not have to refer for a skin biopsy because you should be able to do a simple 4-mm punch biopsy in your office. If you don’t know how to do a skin biopsy, there are workshops given at the both the ACAAI and AAAAI meetings. Urticaria in which the lesions persist beyond 24 hours should definitely be biopsied. Remember, the most important part of the skin biopsy is sending the specimen to a dermatopatholgist familiar with the histologic types of urticaria. It is critical that you give the dermatopatholgist an adequate medical history. 
I find that a skin biopsy is quite helpful and there are three distinct histologic patterns as follows:

1. Group 1: Neutrophilic vasculitis
2. Group 2: Polymorphous perivascular infiltrate
   i. Neutrophils
   ii. Eosinophils
   iii. Mononuclear cells
3. Group 3: Sparse perivascular lymphocytic infiltrate

This can be helpful from a therapeutic standpoint since Group 3 responds nicely to H1 antihistamines, whereas, Group I and Group II will pose a challenge that may require the use of systemic steroids, hydroxychloroquine, cyclosporine and Dapsone.

References
1. Sabroe RA, Fiebiger E, et al. Classification of anti-Fc epsilon R1 and anti-IgE autoantibodies in chronic idiopathic urticaria and correlation with disease activity. J Allergy Clini Immunol 2002;110:492-9.
2. Asero R. Intolerance to nonsteroidal anti-inflammatory drugs might precede by years the onset of chronic urticaria. J Allergy Clin Immunol 2003;111:1095-8.
3. Mehregan DR, Hall MJ, Gibson LE: Urticarial vasculitis: a histopatholigc and clinical review of 72 cases. J Am Acad Dernatikg 1992; 26:441-448, 1992. 
4. Immunology and Allergy Clinics of North America. Ernest N. Charlesworth, MD, guest editor. Volume 15. Number 4. November 1995.

Diagnostic Modalities II: Allergy Diagnostic Testing By William K. Dolen, M.D.

1.  A 36-year-old female presents to you as a new patient because of a recent change in insurance. She wishes to continue allergen immunotherapy for allergic rhinitis, and you have chosen to repeat her skin testing. Modified prick tests are negative to a variety of pollens, danders, mites and other inhalant allergens. The histamine control produced a wheal 5 mm diameter with flare 26 mm diameter. She is astonished because skin testing done only 6 months previously was positive to nearly everything tested. Past medical records are unavailable for review. Which of the following is the most likely explanation for this predicament?

a. She is dermographic.
b. In the previous testing, the test sites were too close together (< 2 cm distance).
c. She has had an immunologic response to immunotherapy that has resulted in suppression of her previously positive tests.
d. She forgot to mention that she has been taking an over-the-counter cold preparation that contains an antihistamine.
e. She was previously tested on the upper back, but you had chosen to repeat testing on her forearms.

2. In which of the following situations would measurement of the total serum IgE level be most appropriate?

a. A 12-year-old male with perennial clear rhinorrhea, nasal and ocular pruritus, and bouts of sneezing.
b. A 42-year-old female with daily urticaria, except when taking antihistamines, for the past 6 months.
c. A 40-year-old male with asthma, a history of recurrent upper lobe pulmonary infiltrates, and a positive skin test to Aspergillus fumigatus.
d. A 4-month-old female with vomiting after feedings
e. A 28-year-old male with chronic rhinitis and a nasal smear positive for eosinophils. Allergy skin tests are negative with appropriately positive controls.

3. A 37-year-old female presents to your practice for evaluation of severe steroid-requiring asthma. At a time when she is not having symptoms, she has a flow-volume loop as depicted in Figure A. Several nights later, she presents to a local emergency room in severe respiratory distress and has a flow-volume loop as in Figure B. Which of the following is the most likely explanation for these findings?

a. Severe intermittent asthma causing variable intrathoracic obstruction
b. A laryngeal polyp causing variable intrathoracic obstruction
c. A bronchogenic cyst causing fixed intrathoracic obstruction
d. Vocal cord dysfunction syndrome causing variable extrathoracic obstruction
e. Vocal cord dysfunction syndrome causing variable intrathoracic obstruction

4. A 16-year-old cheerleader reports daily episodes of shortness of breath and wheezing not related to exercise. On forced expiratory spirometry, the FVC is 105 percent of predicted and the FEV1 is 102 percent of predicted. After nebulization of 2 mg of albuterol, the FEV1 increases to 104 percent of predicted. Review of a diary of symptoms and peak expiratory flow measurements shows no drop in peak flow when symptoms are present and no diurnal variation in peak flow. Symptoms, however, seem to be improved within 45 minutes of using albuterol from a metered dose inhaler. A methacholine challenge is performed, and the concentration of methacholine producing a 20-percent decrease in baseline FEV1 is reported as 15 mg/mL. Which of the following statements is most appropriate?

a. He has asthma, and in accordance with published guidelines should be placed on a controller medication with a short-acting beta adrenergic agonist provided for symptom relief.
b. She has a degree of methacholine reactivity that is present in the normal population. Asthma therapy does not appear to be appropriate, but she should be evaluated for other causes of shortness of breath and wheezing while maintaining close followup.
c. There would be no need to check the test solutions used in order to determine when they had been reconstituted and how they had been stored after reconstitution.
d. The methacholine challenge should be repeated using a dosimeter and a cumulative breath unit protocol.
e. This degree of methacholine reactivity is concerning because of studies that have shown that such individuals are at high risk of developing overt asthma in long-term followup.

5. A 47-year-old male reports progressively severe malaise and fatigue, headaches, dizziness, and difficulty thinking. He notes that symptoms began several months after a large quantity of cleaning solvent was spilled at his workplace. Now, various chemicals, tobacco smoke, polyester clothing, food additives, and chlorinated drinking water aggravate his nearly constant symptoms. His suspicions have been confirmed by provocation-neutralization testing, and he wishes to begin treatment which is to consist of isolation from his modern environment, a rotating diet of natural foods, and injections based on the neutralizing dose. He would like for these injections to be given in your office, which is close to his home. Which of the following statements is the best approach to this situation?
a. Administer the injections as directed by the other physician.
b. Explain that this form of testing and these methods of treatment are not used in your practice. Inform him that he may wish to seek a second opinion before beginning this course of treatment.
c. Perform prick skin testing to inhalants and foods. Perform selected intradermal tests.
d. Tell him that the other physician is clearly a charlatan, that the methods used are experimental, and that the other physician should be reported to the state licensing agency. Refer the patient directly to a psychiatrist.
e. Exclude immunodeficiency by checking lymphocyte subsets by cytofluorometry, as well as mitogen and antigen stimulation.

Answers:

1. A
2. C
3. D
4. B
5. B

 
3. Which is true about RSV infection?
A. Production of IL-11 is decreased in infected cells.
B. The ratio of IFN-gamma/IL-4 is increased in RS- infected cells stimulated by PHA.
C. The PD20 decreases more in atopic than nonatopic individuals who become infected.
D. There are increased numbers of CD19 and CD8 cells.

4. A true statement about cystic fibrosis is:
A. The most common CFTR mutation is abbreviated ΔF508.
B. It is most prevalent in Native Americans.
C. Adults generally have lower sweat chloride concentrations than children.
D. All genotypes are associated with pancreatic insufficiency.

5. Risk factors for asthma death include all but which one?
A. History of respiratory failure requiring ventilation
B. Increase in steroid dosage 50 percent one month before attack
C. Family dysfunction
D. Reaction to separation or loss
E. Hopelessness within one month before attack

Answers:
1. B 
2. D
3. C
4. A
5. B

The Nuts and Bolts of Allergy & Immunology Laboratory Testing
By Rohit K. Katial, M.D.

1. A 35-year-old man presents with a history of episodic subcutaneous swelling, colicky abdominal pain lasting 1-3 days and at times associated with nausea and vomiting. The abdominal pain occurs independent of the swelling. He has also had episodes of throat swelling. General chemistries normal, along with normal ECG, CxR, and UA.

Which of the following tests is most likely to be abnormal in this patient:

A. CH50
B. C3 level
C. C4 level
D. C5 level
E. IgE level

2. A 15-year-old student develops dyspnea and cough. CxR revealed cotton ball densities. Needle aspiration of the densities revealed Aspergillus fumigatus. Past history was also significant for Pseudomonas infections. The patient seemed to recall similar infections in some of his siblings. A DHR assay was run on the patient and his siblings. The results are shown below.

Match the flow pattern most consistent with the appropriate mode of inheritance.

I. ___________ XL CGD
II. ___________ XL CGD carrier
III. ____________ autosomal recessive CGD
 

A)
B)
C)

3. Match the different molecular causes of autosomal recessive SCID with the corresponding cellular profile seen with lymphocyte enumeration.

I. Very low CD3+/CD56+ and CD19+ cells normal or increased
II. Very low CD3+/CD19+/CD56+
III. Very low CD3+/CD19+, normal CD56+
IV. Very low CD3+, normal CD19+/CD56+

A. Jak 3 gene mutations
B. RAG1 or RAG2 mutations
C. IL7R gene mutations
D. ADA mutations

4. A 56-year-old male is golfing on a spring day and begins to experience shortness of breath followed by loss of consciousness. His friend thinks he may have been stung by a bee but is not certain. He is rushed to the emergency room within one hour and is treated with fluid resuscitation, epinephrine, and an H1 blocker. He improves. The emergency room physician consults you to ask if there is any blood work that would help determine if this was anaphylaxis. Which of the following laboratory tests is most likely to be abnormal?

A. Serum histamine
B. Urine histamine
C. Urine N-methyl histamine
D. Serum tryptase
E. Serum chymase

5. Antibodies to polysaccharides in humans are most likely to be which one of the following isotypes?

A. IgG1
B. IgG2
C. IgG3
D. IgG4
E. IgA2


Answers:
1. C
2. I – B
II – C
III – A
3. I – A
II – D
III – B
IV – C
4. D
5. B

Anaphylaxis – Drug, Insect, Food & Vaccine Reactions, Prepared by Mark S. Dykewicz, MD

1. Which of the following is true?
A. Recent food ingestion promotes episodes of exercise-induced anaphylaxis in approximately 50 to 60 percent of patients.
B. H-2 receptor antagonists are more effective than H-1 receptor antagonists in blunting diastolic hypotension induced by intravenous histamine.
C. In most patients, plasma histamine levels remain elevated for 60 to 90 minutes after the onset of anaphylaxis.
D. Serum tryptase levels generally peak within 30 minutes after the onset of IgE-mediated anaphylaxis.

Comparison Questions

2. Associated with elevated serum total IgE levels.

3. Elevated urine histamine levels during acute episodes.

4. Elevated plasma histamine levels between acute episodes.

5. May be associated with increased numbers of mast cells on bone marrow biopsy.

6. May be associated with myelofibrosis on bone marrow biopsy.

7. All of the following are true about aspirin sensitivity EXCEPT:
A. Most aspirin-sensitive patients can tolerate sodium salicylate.
B. Naso-ocular reactions to aspirin are associated with increases in histamine in nasal lavage fluid.
C. Successful "desensitization" to aspirin will not induce tolerance to ibuprofen.
D. Airway reactivity to LTE4 is increased in patients who develop respiratory reactions to aspirin.
E. Acute reactions to aspirin may be associated with elevated serum tryptase levels.

8. Which one of the following is true about stinging insect allergy?
A. There is considerable allergenic cross reactivity between yellow jacket and hornet venom.
B. A skin test concentration of 0.1 ug/ml of honey bee venom may give an irritant reaction.
C. The major allergenic constituent of honey bee venom is mellitin
D. Immunotherapy with whole body extracts of fire ants is ineffective at preventing recurrent anaphylaxis.
E. Wasps typically leave a stinger at a sting site.

9. Which one of the following statements about food reactions is NOT correct?
A. Most patients with cow’s milk allergy will be allergic to goat’s milk.
B. Ragweed allergy is associated with a higher risk of allergy to honeydew.
C. Most patients with allergy to one tree nut will also be allergic to another tree nut.
D. Allergy to cantaloupe is associated with an increased risk for allergy to avocado.
E. Birch pollen allergy is associated with a higher risk of allergy to apple.

Answers:
1. A
2. D
3. C
4. A
5. A
6. A
7. C
8. A
9. C

Topic: Statistics in Clinical Medicine
Prepared by Joyce Hershey, Research Associate,
Walter Reed Army Medical Center, Washington, D.C.

1. A recent study* evaluating commercial in-vitro tests for latex-specific IgE antibodies tested 143 subjects with or without latex allergy (as defined by a combination of history and skin testing to latex — the gold standard) by an in-vitro assay. Here are the results:

o 66 subjects with latex allergy tested positive in the in-vitro assay
o 17 subjects with latex allergy tested negative in the in-vitro assay
o 6 subjects without latex allergy tested positive in the in-vitro assay
o 54 subjects without latex allergy tested negative in the in-vitro assay

Prepare a 2 x 2 table and answer the following questions:

a) What is the sensitivity of the in-vitro assay?

b) What is the specificity of the commercial skin prick test?

c) What is the prevalence of actual disease in this population?

d) What is the positive predictive value (PPV)?

e) What is the negative predictive value?

f) What is the diagnostic accuracy (efficiency)?

g) What is the likelihood ratio for a positive test?

h) What is the likelihood ratio for a negative test?


2. Calculate the PPV of a test that has a sensitivity of 94%, a specificity of 78% for a disease with a population prevalence of 20%.

3. What would the PPV be if the prevalence was 80%?

4. Given the 2 x 2 table below, match the following:

Disease +     Disease -             Total
Test  + a (true positive)   b (false positive)    a + b
Test   - c (false negative)   d (true negative)   c + d
Total   a + c            b + d        a + b + c + d
 

ANSWERS:
1.
a. 79.5%
b. 90%
c. 58%
d. 91.7%
e. 76.1%
f. 83.9%
g. ~8
h. 0.23

2. ~52%
3. ~95%
4.
a. (iv)
b. (v)
c. (i)
d. (ii)
e. (vi)
f. (iii)

*Ownby DR et al. A blinded, multi-center evaluation of two commercial in vitro tests for latex-specific IgE antibodies. Ann Allergy Asthma Immunol. 2000, 84:193-196.

August 6, 2003
Topic: Immunotherapy
1) The purpose of standardized allergen extracts is to:
A. Provide pharmaceutical-grade extracts with minimal lot-to-lot variability that can be interchanged without risk of adverse reactions in immunotherapy
B. Provide extract with clinically irrelevant components removed
C. Lessen the variability in potency from manufacturer-to-manufacturer, and lot-to-lot
D. Assign meaningful potency units to allergen extracts in a manner that reflects the allergen content of extracts
E. Develop a single worldwide system for assigning potency units to allergen extracts

2) To minimize loss in potency, allergen extracts:
A. Should be diluted and stored at room temperature
B. Should be diluted in normal saline with phenol used as a preservative
C. Should be mixed with fungal or dust mite extracts containing significant protease activity
D. Should be stored in the refrigerator in the most concentrated form available
E. Should be standardized

3) The proper maintenance dose of immunotherapy for an individual patient:
A. Must be individualized
B. Requires life-long administration for persistent resolution of symptoms
C. May be determined by titrated intradermal skin testing prior to the start of injections
D. May be determined from published immunotherapy studies using standardized extracts with known major allergen content
E. May be determined by results of quantitative allergen-specific IgE immunoassays performed prior to the start of injections

4) Immunotherapy in asthma:
A. Is contraindicated due to evidence demonstrating minimal efficacy and high prevalence of severe side effects
B. Has been shown to produce symptomatic improvement in double-blind, placebo-controlled trials
C. Primarily benefits the associated rhinitis symptoms
D. Requires lower doses than those needed in rhinitis
E. Produces a clinical effect primarily by augmenting the Th2 cytokine response to lymphocyte stimulation

5) Controversial treatments for allergic diseases include:
A. Autologous urine injection
B. Immunotherapy for allergy mediated by allergen-specific IgG
C. Elimination diets and antifungal therapy for excessive yeast growth
D. Sublingual administration of chemicals to which the patient has been shown to be allergic
E. All of the above

Answers:
1. C
2. D
3. A
4. B
5. E

July 23, 2003
Topic: Aerobiology
Match the allergens with the appropriate characteristic
A. Cyn d 7 from Bermuda grass
B. Pol p 4 from paper wasp
C. Per a 7 from American cockroach
D. Lol p 1 from perennial ryegrass pollen

1. A minor allergen with calcium-binding properties with homology to Bet v 4
2. A tropomyosin with cross-reactivity with shrimp allergen
3. A serine protease
4. A papain-like cysteine protease with beta expansin activity

5. Which of the following statements concerning environmental control of indoor allergens is correct?
A. Tristan de Cunha islanders born at least one year after the removal of all cats from the island show no sensitization to Fel d 1
B. Synthetic fiber pillows will show slower accumulation and lower absolute levels of Der p 1 than down feather pillows
C. A meta-analysis of 23 house dust mite control measures in the management of asthma revealed no impact on PEFR or standardized asthma symptom improvement
D. In European homes with atopic children, mold growth could not be correlated with PEFR variability
E. None of the above

6. Which of the following statements concerning arthropod inhalant allergy is FALSE
A. In the absence of special cleaning efforts, German cockroach extermination does not impact on Bla g 1 or Bla g 2 levels in single-family homes within the first 6 months
B. The majority of airborne particles carrying Bla g 1 in undisturbed rooms are under 10 microns in size
C. Reducing the mean daily relative humidity below 50%, even with levels greater than 50% for 2-8 hours, restricts D. farinae population growth
D. Large local reactions to mosquito bites in small children appear to be mediated by IgE and IgG conjointly

Cross-reactivity between each of the following pairs is due to which substances?
A. Zucchini and melon
B. Birch and celery
C. Both
D. Neither

7. Profillin and carbohydrate determinants
8. Bet v 1, Bet v 2, and 40-60 kilodalton molecules

Match the correct fungal allergens with the following statements
A. Enolase
B. Acid ribosomal protein P2
C. Both
D. Neither

9. Major Cladosporium and Alernaria allergens Cla h 6 and Alt a 11
10. Highly conserved human SLE antigen which is Alt a 6 and Cla h 4

Answers:
1. A
2. C
3. B
4. D
5. C
6. B
7. A
8. B
9. A
10. B

Topic: Immunohematologic Disorders

1) A 19-year-old male student presents to your office complaining of weakness and exertional shortness of breath. Approximately 10 days ago, he had the onset of a sore throat, fever, and headache. Physical examination: pale young man. Throat injected. Cervical adenopathy. Spleen palpable just below the left costal margin. Lab: Hct=19, WBC=6000. Atypical lymphocytes on blood smear. Some red cells clumped. UA – red brown color, Hemoglobin +. DAT +. Which of the following is likely NOT to be present in this patient?

A. The DAT test will be positive for only C3.
B. The cold agglutinin may show specificity for the small I antigen.
C. The cold agglutinin is usually a monoclonal IgM.
D. Cold agglutinin titer may be positive only with cord red blood cells.
E. Haptoglobin will be low.

2) Match the following autoantibodies with the clinical disorder:
 

3) Match the following clinical features with the clinical disorders:

4) All of the following are true regarding patients with essential mixed cryoglobulinemia EXCEPT:

A. The serum C4 is elevated.
B. Rheumatoid factor is positive.
C. HCV RNA is frequently present in the serum.
D. Presence of glomerulonephritis.
E. IgM-IgG is most often found in the cryoglobulin.

5) Match the following features with the clinical disorders below:

6) All of the following are true regarding immune thrombocytopenia EXCEPT:

A. Can be a presenting feature of systemic lupus erythematosus.
B. Heparin induced thrombocytopenia may be associated with life threatening vascular occlusion.
C. Can occur in HIV infection.
D. In ITP, the anti-platelet antibody is directed to platelet factor 4.
E. In young children, ITP is usually acute and self limited.

Answers: 1) C
2) 1=B, 2=A, 3=D, 4=C, 5=E
3) 1=C, 2=A, 3=D, 4=E, 5=B
4) A
5) 1=C, 2=E, 3=A, 4=B, 5=D
6) D

Topic: Immunopharmacology II

Of the following statements (#1-#9), select the appropriate answer

1) Is a purine analog related to 6-mercaptopurine
2) Does not appear to have major effects on T cell proliferation and established delayed hypersensitivity reactions
3) Liver function tests should be performed regularly to detect hepatotoxicity
4) Is an alkylating agent related to nitrogen mustard
5) Granulocytopenia is the most common dose-related hematologic toxicity
6) Is an absolute contraindication in pregnant women
7) Hair loss is common
8) Patients with a history of daily administration require life-long surveillance for bladder cancer
9) The principle pathway of detoxification of the drug(s) is inhibited by allopurinol

10) Match the interferon or interleukin listed below with the appropriate clinical indication

Answers:
1) A
2) A
3) A
4) B
5)C
6) B
7) B
8) B
9) A
10) A=4, B=5, C=1, D=1, E=2, F=1, G=4, H=3, I=5, J=6

April 30, 2003
Topic: Allergic Skin Diseases

1) Which one of the following is a pre-formed skin mast cell mediator
A. LTC4
B. Chymotrypsin
C. PGD2
D. AGEPC (platelet activating factor)
E. LTB4

2) Which of the following symptoms is LEAST characteristic of urticarial vasculitis
A. Pruritis
B. Burning
C. Stinging
D. Soreness
E. Arthralgia

3) Which statement concerning urticaria and the role of foods is TRUE
A. Urticaria is frequently caused by a combination of foods that individually will not cause hives
B. The season of the year may contribute to which foods cause hives
C. Pork should be eliminated from the diet in urticarial patients allergic to pigweed
D. Foods that cause urticaria are usually obvious to the patient

4) There are 2 types of allergic reactions to latex: IgE mediated allergy and allergic contact dermatitis. Which of the following agents would not be expected to be positive on patch testing in a patient with an allergic contact dermatitis to latex
A. Mercapto mix
B. Parabens
C. Black rubber mix
D. Mercaptobenzothiazole (MBT)
E. Thiuram mix

5) Which of the following statements regarding atopic dermatitis is FALSE
A. Atopic dermatitis is ‘an itch which rashes, rather than a rash that itches’
B. Like allergic rhinitis and asthma, AD is characterized by an imbalance in Th1 CD4+ lymphocytes that produce certain ‘pro-allergic’ cytokines
C. Staphylococcal exotoxins may act as superantigens in atopic dermatitis patients and result in the activation of as many as 40 percent of the lymphocytes without the presence of a protein antigen
D.Tacrolimus inhibits all of the following cytokines: IL-2, IL-3, IL-4, IL-5, GM-CSF, TNF-(, IFN-(
E. Tacrolimus decreases the expression of intracellular attachment molecule ICAM-1

Answers:
1) B
2) D
3) D
4) B
5) B

Topic: Autoimmune Diseases

Topic: Immunodeficiencies

1) A 2-year-old boy and his 3-year-old sister both suffer from repeated infections of the skin, lymph nodes, and gingiva. White blood cell counts repeatedly show extremely elevated values, even when their infections subside. The mother and father are both healthy, and there is no family history of similar infections. What diagnosis is most consistent with these findings?

A. Complement component 6 deficiency
B. Selective IgA deficiency
C. Bruton’s tyrosine kinase deficiency
D. Leukocyte adhesion deficiency
E. Chronic granulomatous disease

2) A 10-month-old girl develops empyema of the lung and fistulous tract through the diaphragm, leading to peritonitis. Here serum immunoglobulins are moderately elevated, she has a 10mm Candida skin test reaction at 48 hours, normal NBT test, and a total hemoltyic complement (CH50) of 0. What laboratory test would reveal her diagnosis?

A. Mitogen stimulation of T cells
B. Adhesion glycoproteins of neutrophils
C. C3 complement component
D. Bacteriophage (fX 174) immunization
E. Immunoglobulin subclass measurement

3) Which patient has X-linked agammaglobulinemia

A. Patient 1
B. Patient 2
C. Patient 3
D. Patient 4

4) A kindergarten school teacher is the mother of a 4-year-old boy and a 4-month-old boy, both of whom attend a nursery school during her work days. The mother has had two bouts of pneumonia within the last year, which coincide with the oldest boy’s fever and upper respiratory infection; all documented by a physician. Upon a repeated family illness, serum immunoglobulin levels, functional antibody tests, and lymphocyte subsets are obtained:

The correct diagnosis of this family’s problem is:
A. The 4-month-old has transient hypogammaglobulinemia of childhood
B. All of the family members have normal immunity
C. The 4-month-old has X-linked agammaglobulinemia (Bruton’s disease)
D.The 4-month-old has a pre-malignant disease of lymphocytes
E. The mother has a B cell deficiency

5) In terms of possible treatment with IVIG, your choice would be:
A. Treat the 4-month-old with 2-4 months of IVIG
B. Give the boys and the mother a therapeutic trial of IVIG
C. Initiate life-long IVIG for the 4-month-old
D. Give no IVIG but refer the 4-month-old to an oncologist
E. Consider giving IVIG to the mother

Answers:
1) D
2) C
3) D
4) E
5) E

March 5, 2003

Topic: Autoimmune Diseases

1) Immune mechanisms underlying type 1 diabetes include all of the following EXCEPT:
A. Antigen recognition and co-stimulation
B. T cell (CD8) cytotoxicity
C. Beta cell apoptosis
D. Immune complex injury of the pancreatic islets
E. Autoantibody production

2) All these autoimmune diseases are similar in their pathogenesis except:
A. Type 1 diabetes
B. Addison’s disease
C. Atrophic gastritis
D. Grave’s disease
E. Hashimoto’s thyroiditis

3) Patients with autoimmune thyroid disease display immune responses to which of the following autoantigens:
A. Na=H+ ATPase
B. Glutamic acid decarboxylase
C. Myelin basic protein
D. Peroxidase enzyme
E. Insulin

4) Fas antigen mediated apoptosis is:
A. Mediated by soluble TNF
B. Different in morphology from necrosis
C. Occurs only in diabetes
D. Restricted to immune cells
E. Mediated by CD4 cells