Allergy and Clinical Immunology E-Book

• Herausgeber: John Wiley & Sons
• Kategorie: Fachliteratur
• Serie: Mount Sinai Expert Guides
• Sprache: Englisch

Seitenzahl: 734

Veröffentlichungsjahr: 2015

Beschreibung

Mount Sinai Expert Guides: Allergy and Clinical Immunology will provide trainees in allergy and immunology with an extremely clinical and accessible handbook covering the major disorders and symptoms, their diagnosis and clinical management.

Perfect as a point-of-care resource on the hospital wards and also as a refresher for board exam preparation, the focus throughout is on providing rapid reference, essential information on each disorder to allow for quick, easy browsing and assimilation of the must-know information. All chapters follow a consistent template including the following features:

• An opening bottom-line/key points section
• Classification, pathogenesis and prevention of disorder
• Evidence-based diagnosis, including relevant algorithms, laboratory and imaging tests, and potential pitfalls when diagnosing a patient
• Disease management including commonly used medications with dosages, management algorithms and how to prevent complications
• How to manage special populations, ie, in pregnancy, children and the elderly
• The very latest evidence-based results, major society guidelines and key external sources to consult

In addition, the book comes with a companion website housing extra features such as case studies with related questions for self-assessment, key patient advice and ICD codes. Each guide also has its own mobile app available for purchase, allowing you rapid access to the key features wherever you may be.

If you're specialising in allergy and immunology and require concise, practical and clinical guidance from one of the world's leading institutions in this field, then this is the perfect book for you.

This title is also available as a mobile App from MedHand Mobile Libraries. Buy it now from iTunes, Google Play or the MedHand Store.

Leseprobe

CONTENTS

Cover

Title page

Contributors

Series Foreword

Preface

List of Abbreviations

About the Companion Website

PART 1: Allergy

CHAPTER 1: Atopic Dermatitis in Infants and Young Children

Section 1: Background

Section 2: Prevention

Section 3: Diagnosis (Algorithm 1.1)

Section 4: Treatment (Algorithm 1.2)

Section 5: Special populations

Section 6: Prognosis

Section 7: Reading list

Section 8: Guidelines

Section 9: Evidence

Section 10: Images

CHAPTER 2: Atopic Dermatitis in Teenagers and Adults

Section 1: Background

Section 2: Prevention

Section 3: Diagnosis (Algorithm 2.1)

Section 4: Treatment (Algorithm 2.2)

Section 5: Special populations

Section 6: Prognosis

Section 7: Reading list

Section 8: Guidelines

Section 9: Evidence

Section 10: Images

CHAPTER 3: Contact Dermatitis

Section 1: Background

Section 2: Prevention

Section 3: Diagnosis (Algorithm 3.1)

Section 4: Treatment (Algorithm 3.2)

Section 5: Special populations

Section 6: Prognosis

Section 7: Reading list

Section 8: Guidelines

Section 9: Evidence

Section 10: Images

CHAPTER 4: Allergic Rhinitis

Section 1: Background

Section 2: Prevention

Section 3: Diagnosis

Section 4: Treatment

Section 5: Special populations

Section 6: Prognosis

Section 7: Reading list

Section 8: Guidelines

Section 9: Evidence

Section 10: Images

CHAPTER 5: Non-Allergic Rhinitis

Section 1: Background

Section 2: Prevention

Section 3: Diagnosis (Algorithm 5.1)

Section 4: Treatment

Section 5: Special populations

Section 6: Prognosis

Section 7: Reading list

Section 8: Guidelines

Section 9: Evidence

Section 10: Images

CHAPTER 6: Sinusitis

Section 1: Background

Section 2: Prevention

Section 3: Diagnosis

Section 4: Treatment

Section 5: Special populations

Section 6: Prognosis

Section 7: Reading list

Section 8: Guidelines

Section 9: Evidence

Section 10: Images

CHAPTER 7: Allergic Diseases of the Eye

Section 1: Background

Section 2: Prevention

Section 3: Diagnosis (Algorithm 7.1)

Section 4: Treatment (Algorithm 7.2)

Section 5: Special populations

Section 6: Prognosis

Section 7: Reading list

Section 8: Guidelines

Section 9: Evidence

Section 10: Images

CHAPTER 8: Tests for Assessing Asthma

Section 1: Background

Section 2: Prevention

Section 3: Diagnosis

Section 4: Treatment

Section 5: Special populations

Section 6: Prognosis

Section 7: Reading list

Section 8: Guidelines

Section 9: Evidence

Section 10: Images

CHAPTER 9: Asthma in Infants and Children

Section 1: Background

Section 2: Prevention

Section 3: Diagnosis

Section 4: Treatment

Section 5: Special populations

Section 6: Prognosis

Section 7: Reading list

Section 8: Guidelines

Section 9: Evidence

Section 10: Images

CHAPTER 10: Asthma in Teenagers and Adults

Section 1: Background

Section 2: Prevention

Section 3: Diagnosis

Section 4: Treatment

Section 5: Special populations

Section 6: Prognosis

Section 7: Reading list

Section 8: Guidelines

Section 9: Evidence

Section 10: Images

CHAPTER 11: Evaluation of Cough

Section 1: Background

Section 2: Prevention

Section 3: Diagnosis

Section 4: Treatment

Section 5: Special populations

Section 6: Prognosis

Section 7: Reading list

Section 8: Guidelines

Section 9: Evidence

Section 10: Images

CHAPTER 12: Exercise-Induced Asthma

Section 1: Background

Section 2: Prevention

Section 3: Diagnosis

Section 4: Treatment

Section 5: Special populations

Section 6: Prognosis

Section 7: Reading list

Section 8: Guidelines

Section 9: Evidence

Section 10: Images

CHAPTER 13: Occupational Asthma

Section 1: Background

Section 2: Prevention

Section 3: Diagnosis

Section 4: Treatment

Section 5: Special populations

Section 6: Prognosis

Section 7: Reading list

Section 8: Guidelines

Section 9: Evidence

Section 10: Images

CHAPTER 14: Status Asthmaticus and Pending Pulmonary Failure

Section 1: Background

Section 2: Prevention

Section 3: Diagnosis

Section 4: Treatment (Algorithm 14.1)

Section 5: Special populations

Section 6: Prognosis

Section 7: Reading list

Section 8: Guidelines

Section 9: Evidence

Section 10: Images

CHAPTER 15: Evaluation of Food Allergy

Section 1: Background

Section 2: Prevention

Section 3: Diagnosis (Algorithm 15.1)

Section 4: Treatment

Section 5: Special populations

Section 6: Prognosis

Section 7: Reading list

Section 8: Guidelines

Section 9: Evidence

Section 10: Images

CHAPTER 16: Oral Allergy Syndrome

Section 1: Background

Section 2: Prevention

Section 3: Diagnosis (Algorithm 16.1)

Section 4: Treatment (Algorithm 16.2)

Section 5: Special populations

Section 6: Prognosis

Section 7: Reading list

Section 8: Guidelines

Section 9: Evidence

Section 10: Images

CHAPTER 17: Food Allergy and Atopic Dermatitis

Section 1: Background

Section 2: Prevention

Section 3: Diagnosis (Algorithm 17.1)

Section 4: Treatment

Section 5: Special populations

Section 6: Prognosis

Section 7: Reading list

Section 8: Guidelines

Section 9: Evidence

Section 10: Images

CHAPTER 18: Food Protein-Induced Enterocolitis Syndrome

Section 1: Background

Section 2: Prevention

Section 3: Diagnosis (Algorithm 18.1)

Section 4: Treatment (Algorithm 18.2)

Section 5: Special populations

Section 6: Prognosis

Section 7: Reading list

Section 8: Guidelines

Section 9: Evidence

Section 10: Images

CHAPTER 19: Food Protein-Induced Proctocolitis Syndrome

Section 1: Background

Section 2: Prevention

Section 3: Diagnosis (Algorithm 19.1)

Section 4: Treatment (Algorithm 19.2)

Section 5: Special populations

Section 6: Prognosis

Section 7: Reading list

Section 8: Guidelines

Section 9: Evidence

Section 10: Images

CHAPTER 20: Eosinophilic Esophagitis

Section 1: Background

Section 2: Prevention

Section 3: Diagnosis (Algorithm 20.1)

Section 4: Treatment (Algorithm 20.2)

Section 5: Special populations

Section 6: Prognosis

Section 7: Reading list

Section 8: Guidelines

Section 9: Evidence

Section 10: Images

CHAPTER 21: Eosinophilic Gastroenteritis

Section 1: Background

Section 2: Prevention

Section 3: Diagnosis (Algorithm 21.1)

Section 4: Treatment (Algorithm 21.2)

Section 5: Special populations

Section 6: Prognosis

Section 7: Reading list

Section 8: Guidelines

Section 9: Evidence

Section 10: Images

CHAPTER 22: Food Allergy-Induced Anaphylaxis

Section 1: Background

Section 2: Prevention

Section 3: Diagnosis

Section 4: Treatment (Algorithm 22.1)

Section 5: Special populations

Section 6: Prognosis

Section 7: Reading list

Section 8: Guidelines

Section 9: Evidence

Section 10: Images

CHAPTER 23: Adverse Reactions to Food and Drug Additives

Section 1: Background

Section 2: Prevention

Section 3: Diagnosis (Algorithm 23.1)

Section 4: Treatment (Algorithm 23.2)

Section 5: Special populations

Section 6: Prognosis

Section 7: Reading list

Section 9: Guidelines

Section 9: Evidence

Section 10: Images

CHAPTER 24: Celiac Disease

Section 1: Background

Section 2: Prevention

Section 3: Diagnosis (Algorithm 24.1)

Section 4: Treatment (Algorithm 24.2)

Section 5: Special populations

Section 6: Prognosis

Section 7: Reading list

Section 8: Guidelines

Section 9: Evidence

Section 10: Images

CHAPTER 25: Oral Food Challenges (Procedure)

Section 1: Indications

Section 2: Procedure

Section 3: Management of complications

Section 4: Follow-up

Section 5: Reading list

Section 6: Guidelines

Section 7: Evidence

Section 8: Images

CHAPTER 26: Acute Urticaria

Section 1: Background

Section 2: Prevention

Section 3: Diagnosis

Section 4: Treatment (Algorithm 26.1)

Section 5: Special populations

Section 6: Prognosis

Section 7: Reading list

Section 8: Guidelines

Section 9: Evidence

Section 10: Images

CHAPTER 27: Chronic Urticaria

Section 1: Background

Section 2: Prevention

Section 3: Diagnosis (Algorithm 27.1)

Section 4: Treatment (Algorithm 27.2)

Section 5: Special populations

Section 6: Prognosis

Section 7: Reading list

Section 8: Guidelines

Section 9: Evidence

Section 10: Images

CHAPTER 28: Hereditary Angioedema

Section 1: Background

Section 2: Prevention

Section 3: Diagnosis

Section 4: Treatment

Section 5: Special populations

Section 6: Prognosis

Section 7: Reading list

Section 8: Guidelines

Section 9: Evidence

Section 10: Images

CHAPTER 29: Anaphylaxis

Section 1: Background

Section 2: Prevention

Section 3: Diagnosis

Section 4: Treatment (Algorithm 29.2)

Section 5: Special populations

Section 6: Prognosis

Section 7: Reading list

Section 8: Guidelines

Section 9: Evidence

Section 10: Images

CHAPTER 30: Mastocytosis

Section 1: Background

Section 2: Prevention

Section 3: Diagnosis

Section 4: Treatment

Section 5: Special populations

Section 6: Prognosis

Section 7: Reading list

Section 8: Guidelines

Section 9: Evidence

Section 10: Images

CHAPTER 31: Insect Sting Allergy

Section 1: Background

Section 2: Prevention

Section 3: Diagnosis (Algorithm 31.1)

Section 4: Treatment (Algorithm 31.2)

Section 5: Special populations

Section 6: Prognosis

Section 7: Reading list

Section 8: Guidelines

Section 9: Evidence

Section 10: Images

CHAPTER 32: Latex Allergy

Section 1: Background

Section 2: Prevention

Section 3: Diagnosis

Section 4: Treatment

Section 5: Special populations

Section 6: Prognosis

Section 7: Reading list

Section 8: Guidelines

Section 9: Evidence

Section 10: Images

CHAPTER 33: Allergy to Antibiotics

Section 1: Background

Section 2: Prevention

Section 3: Diagnosis (Algorithm 33.1)

Section 4: Treatment

Section 5: Special populations

Section 6: Prognosis

Section 7: Reading list

Section 8: Guidelines

Section 9: Evidence

Section 10: Images

CHAPTER 34: Allergy to Non-Antibiotic Drugs

Section 1: Background

Section 2: Prevention

Section 3: Diagnosis (Algorithm 34.1)

Section 4: Treatment

Section 5: Special populations

Section 6: Prognosis

Section 7: Reading list

Section 8: Guidelines

Section 9: Evidence

Section 10: Images

CHAPTER 35: Drug Desensitization

Section 1: Background

Section 2: Prevention

Section 3: Diagnosis

Section 4: Treatment

Section 5: Special populations

Section 6: Prognosis

Section 7: Reading list

Section 8: Guidelines

Section 9: Evidence

Section 10: Images

CHAPTER 36: Immunotherapy (Procedure)

Section 1: Background

Section 2: Prevention

Section 3: Diagnosis

Section 4: Treatment (Algorithm 36.1)

Section 5: Special populations

Section 6: Prognosis

Section 7: Reading list

Section 8: Guidelines

Section 9: Evidence

Section 10: Images

CHAPTER 37: Allergy Testing (Procedures: Skin Testing; Blood Tests)

Section 1: Skin prick testing

Section 2: Intradermal (intracutaneous) skin testing

Section 3: Patch testing (epicutaneous patch test)

Section 4: Serum tests for detection of specific IgE

Section 5: Reading list

Section 6: Guidelines

Section 7: Evidence

Section 8: Images

PART 2: Clinical Immunology

CHAPTER 38: Evaluating the Child with Recurrent Infections

Section 1: Background

Section 2: Prevention

Section 3: Diagnosis (Algorithm 38.1)

Section 4: Treatment

Section 5: Special populations

Section 6: Prognosis

Section 7: Reading list

Section 8: Guidelines

Section 9: Evidence

Section 10: Images

CHAPTER 39: Evaluating the Adult with Recurrent Infections

Section 1: Background

Section 2: Prevention

Section 3: Diagnosis (Algorithm 39.1)

Section 4: Treatment

Section 5: Special populations

Section 6: Prognosis

Section 7: Reading list

Section 8: Guidelines

Section 9: Evidence

Section 10: Images

CHAPTER 40: Antibody Deficiencies

Section 1: Background

Section 2: Prevention

Section 3: Diagnosis

Section 4: Treatment

Section 5: Special populations

Section 6: Prognosis

Section 7: Reading list

Section 8: Guidelines

Section 9: Evidence

Section 10: Images

CHAPTER 41: Selective IgA Deficiency

Section 1: Background

Section 2: Prevention

Section 3: Diagnosis (Algorithm 41.1)

Section 4: Treatment (Algorithm 41.2)

Section 5: Special populations

Section 6: Prognosis

Section 7: Reading list

Section 8: Guidelines

Section 9: Evidence

Section 10: Images

CHAPTER 42: Severe Combined Immunodeficiency

Section 1: Background

Section 2: Prevention

Section 3: Diagnosis

Section 4: Treatment

Section 5: Special populations

Section 6: Prognosis

Section 7: Reading list

Section 8: Guidelines

Section 9: Evidence

Section 10: Images

CHAPTER 43: Wiskott–Aldrich Syndrome

Section 1: Background

Section 2: Prevention

Section 3: Diagnosis

Section 4: Treatment

Section 5: Special populations

Section 6: Prognosis

Section 7: Reading list

Section 8: Guidelines

Section 9: Evidence

Section 10: Images

CHAPTER 44: DiGeorge Syndrome

Section 1: Background

Section 2: Prevention

Section 3: Diagnosis

Section 4: Treatment

Section 5: Special populations

Section 6: Prognosis

Section 7: Reading list

Section 8: Guidelines

Section 9: Evidence

Section 10: Images

CHAPTER 45: Chronic Mucocutaneous Candidiasis

Section 1: Background

Section 2: Prevention

Section 3: Diagnosis (Algorithm 45.1)

Section 4: Treatment (Algorithm 45.2)

Section 5: Special populations

Section 6: Prognosis

Section 7: Reading list

Section 8: Guidelines

Section 9: Evidence

Section 10: Images

CHAPTER 46: X-linked Immune Dysregulation with Polyendocrinopathy (IPEX) Syndrome

Section 1: Background

Section 2: Prevention

Section 3: Diagnosis (Algorithm 46.1)

Section 4: Treatment

Section 5: Special populations

Section 6: Prognosis

Section 7: Reading list

Section 8: Guidelines

Section 9: Evidence

Section 10: Images

CHAPTER 47: Autoimmune Lymphoproliferative Syndrome

Section 1: Background

Section 2: Prevention

Section 3: Diagnosis

Section 4: Treatment

Section 5: Special populations

Section 6: Prognosis

Section 7: Reading list

Section 8: Guidelines

Section 9: Evidence

Section 10: Images

CHAPTER 48: Hyper IgE Syndrome

Section 1: Background

Section 2: Prevention

Section 3: Diagnosis (Algorithm 48.1)

Section 4: Treatment (Algorithm 48.2)

Section 5: Special populations

Section 6: Prognosis

Section 7: Reading list

Section 8: Guidelines

Section 9: Evidence

Section 10: Images

CHAPTER 49: Deficiencies of Complement

Section 1: Background

Section 2: Prevention

Section 3: Diagnosis

Section 4: Treatment

Section 5: Special populations

Section 6: Prognosis

Section 7: Reading list

Section 8: Guidelines

Section 9: Evidence

Section 10: Images

CHAPTER 50: Phagocytic Cell Disorders

Section 1: Background

Section 2: Prevention

Section 3: Diagnosis

Section 4: Treatment

Section 5: Special populations

Section 6: Prognosis

Section 7: Reading list

Section 8: Guidelines

Section 9: Evidence

Section 10: Images

CHAPTER 51: Human Immunodeficiency Virus Infection in Infants, Children, and Adolescents

Section 1: Background

Section 2: Prevention

Section 3: Diagnosis (Algorithm 51.1)

Section 4: Treatment

Section 5: Special populations

Section 6: Prognosis

Section 7: Reading list

Section 8: Guidelines

Section 9: Evidence

Section 10: Images

CHAPTER 52: Human Immunodeficiency Virus Infection in Adults

Section 1: Background

Section 2: Prevention

Section 3: Diagnosis (Algorithm 52.1)

Section 4: Treatment

Section 5: Special populations

Section 6: Prognosis

Section 7: Reading list

Section 8: Guidelines

Section 9: Evidence

Section 10: Images

CHAPTER 53: Infections in the Compromised Host

Section 1: Background

Section 2: Prevention

Section 3: Diagnosis

Section 4: Treatment

Section 5: Special populations

Section 6: Prognosis

Section 7: Reading list

Section 8: Guidelines

Section 9: Evidence

Section 10: Images

Index

End User License Agreement

List of Tables

Chapter 08

Table 8.1 Classifying asthma severity and initiating treatment in youths ≥12 years of age and adults. Assessing severity and initiating treatment for patients who are not currently taking long-term medications. The table demonstrates that spirometric impairment is an important determinant of asthma severity.

Chapter 09

Table 9.1 Classifying asthma severity and initiating treatment in children 5–11 years of age, who are not currently taking long-term control medication.

Chapter 13

Table 13.1 Principal agents causing immunologic occupational asthma.

Table 13.2 Common causes of irritaion in various situations.

Chapter 18

Table 18.1 Empirical recommendations for dietary management of food protein-induced enterocolitis (FPIES). No controlled trials have been performed to determine optimal timing of introduction in infants and toddlers with FPIES.

Chapter 25

Table 25.1 Oral food challenge symptom scoring.

Chapter 48

Table 48.1 A clinical scoring system to include manifestations of hyper-IgE syndrome (HIES) developed by the Natonal Institutes of Health (NIH) is available. A score >40 is suggestive of AD-HIES

Chapter 49

Table 49.1 Inherited complement deficiencies and clinical associations.

List of Illustrations

Chapter 01

Algorithm 1.1 Diagnosis of atopic dermatitis

Algorithm 1.2 Management of atopic dermatitis

Figure 1.1 A child with multiple food allergies and severe persistent atopic dermatitis (AD) with acute exacerbation due to

Staphylococcus aureus

superinfection. Note the diffuse erythroderma and open sores.

Figure 1.2 AD chronic lesions of skin hypertrophy, lichenification, hyperpigmentation, and xerosis.

Chapter 02

Algorithm 2.1  Diagnosis of atopic dermatitis

Algorithm 2.2  Treatment of atopic dermatitis

Figure 2.1 SCORAD: Scoring Atopic Dermatitis, is a scoring system for assessment of atopic dermatitis. Availalbe at http://www.fondation-dermatite-atopique.org/en/healthcare-professionals-space/scorad

Chapter 03

Algorithm 3.1 Diagnosis of contact dermatitis

Algorithm 3.2 Treatment of contact dermatitis

Figure 3.1 Contact dermatitis to nickel from belt buckle.

Figure 3.2 Contact dermatitis to propylene glycol, a preservative used in steroid creams. Consider this diagnosis if patient is not responding to treatment.

Figure 3.3 Contact dermatitis to Bronopol.

Chapter 05

Algorithm 5.1 Diagnosis of non-allergic rhinitis

Chapter 06

Figure 6.1 Nasal endoscopy of left middle meatus with purulent drainage noted in infundibulum. Patient had an acute maxillary sinusitis.

Figure 6.2 Acute sinusitis. Non-contrast CT scan of the sinuses with an acute left odontogenic maxillary sinusitis. Patient has an air–fluid level in the left maxillary sinusitis with a dental implant placed into the floor of the maxillary sinus. Patient required maxillary antrostomy to clear the infection.

Figure 6.3 Nasal endoscopy of patient with nasal polyps and complete nasal airway obstruction. This patient had complaints of anosmia and nasal congestion.

Figure 6.4 Chronic rhinosinusitis with polyposis. Non-contrast CT scan of the sinuses of patient in Figure 6.3 demonstrates expansile polyposis of the right sinonasal cavity. The arrow points to area of polyp extension from ethmoid cavity into the orbit. In these cases the periorbita of the eye is usually not violated; however, caution is needed during surgical clearance of polyps in this area to avoid risk of intraorbital injury.

Chapter 07

Algorithm 7.1 Diagnosis of allergic diseases of the eye

Algorithm 7.2 Management of allergic diseases of the eye

Figure 7.1 Vernal conjunctivitis – cobblestone papillae cover the superior tarsal conjunctiva. From Rubenstein JB, Tannan A. Conjunctivitis: infectious and noninfectious. In Yanoff M, Duker JS, eds. Ophthalmology. 4th ed. St. Louis, MO, Mosby Elsevier; 2013:chap 4.6. Reproduced with permission.

Chapter 08

Figure 8.1 (A) shows the exhalation portion of a flow volume loop and demonstrates an obstructive pattern in contrast to normal spirometry. In obstruction, there is “scooping” of the descending limb of the flow volume loop attributable to a decrease in the FEV

1

/FVC ratio. (B) demonstrates that in asthma, obstruction is generally reversible in response to bronchodilator. In the figure, there is less “scooping” post-bronchodilator and increase in flows. The increase in volume noted corresponds to an increase in forced vital capacity (FVC).

Chapter 09

Figure 9.1 Stepwise approach for managing asthma in children 5–11 years of age.

Chapter 11

Algorithm 11.1 Diagnosis of acute cough for patients ≥15 years of age with cough lasting <3 weeks

Algorithm 11.2 Diagnosis of subacute cough for patients ≥15 years of age with cough lasting 3–8 weeks

Algorithm 11.3 Diagnosis of chronic cough algorithm for patients ≥15 years of age with cough lasting >8 weeks

Chapter 14

Algorithm 14.1 Management of asthma exacerbations: emergency department and hospital-based care

Chapter 15

Algorithm 15.1  General approach to diagnosis of food allergy

Chapter 16

Algorithm 16.1  Diagnosis of oral allergy syndrome (OAS)

Algorithm 16.2  Management of oral allergy syndrome

Chapter 17

Algorithm 17.1 Diagnosis of atopic dermatitis

Chapter 18

Algorithm 18.1 Diagnosis of food protein-induced enterocolitis syndrome (FPIES)

Algorithm 18.2 Management of FPIES

Chapter 19

Algorithm 19.1 Diagnosis of food protein-induced proctocolitis

Algorithm 19.2 Management of food protein-induced proctocolitis

Chapter 20

Algorithm 20.1 Diagnosis of eosinophilic esophagitis (EoE)Note: EGD, esophagogastroduodenoscopy; GERD, gastroesophageal reflux disease; HPF, high power field; PPI-REE, proton pump inhibitor-responsive esophageal eosinophilia. Note that whenever remission is mentioned, it refers to clinical and histologic remission.

Algorithm 20.2 Treatment of EoENote: APT, atopy patch tests; EGD, esophagogastroduodenoscopy; SPT, skin prick tests. Note that whenever remission is mentioned, it refers to clinical and histologic remission.

Figure 20.1 Inflammatory features of eosinophilic esophagitis (EoE) on endoscopy. Patients with EoE can present with furrows (A), white plaques (B), or white plaques aligning along the furrows (C) on endoscopy.

Figure 20.2 Fibrostenotic features of EoE on endoscopy. Rings (A), esophageal stricture (B), or esophageal shearing (C) can be present in patients with advanced EoE on endoscopy.

Chapter 21

Algorithm 21.1 Diagnosis of eosinophilic gastroenteritis (EG)Note: EGD, esophagogastroduodenoscopy; GI, gastrointestinal; HES, hypereosinophilic syndrome; IBS, irritable bowel syndrome.

Algorithm 21.2 Treatment of EGDNote: APT, atopy patch tests; EGD, esophagogastroduodenoscopy; SPT, skin prick tests. Note that whenever remission is mentioned, it refers to clinical and histologic remission.

Figure 21.1 Possible features of eosinophilic gastroenteritis (EG) on endoscopy include multiple erosions of the gastric mucosa (A) or multiple gastric pseudopolyps in advanced cases (B).

Chapter 22

Algorithm 22.1 Management of anaphylaxis

Chapter 23

Algorithm 23.1  Diagnosis of adverse reactions to food and drug additives

Algorithm 23.2  Management of adverse reactions to food and drug additives

Chapter 24

Algorithm 24.1 Diagnosis of celiac diseaseNote: CD, celiac disease; DGP IgG, deamidated gliadin peptide IgG; TTG-IgA, tissue transglutaminase IgA titer; TTG-IgG, tissue transglutaminase IgG titer.

Algorithm 24.2 Management of celiac diseaseNote: CD, celiac disease; GFD, gluten-free diet.

Figure 24.1 Histopathologic features of an intestinal biopsy from a patient with celiac disease (hematoxylin and eosin stain, magnification 100×): Blunted villi, crypt hyperplasia, mononuclear infiltration of the lamina propria, and intraepithelial lymphocytic infiltration. The latter is made visually clear in the inset (magnification 400×).

Chapter 26

Algorithm 26.1 Treatment of acute urticaria

Figure 26.1 Superficial urticarial lesions often have central pallor and raised erythematous edematous borders, but can vary in size, shape, and color. From Habif TP. Clinical Dermatology: A Color Guide to Diagnosis and Therapy. 5th edition. 2010 Philadelphia, PA, Mosby/Elsevier. Reproduced with permission.

Figure 26.2 Confluent urticaria. From Habif TP. Clinical Dermatology: A Color Guide to Diagnosis and Therapy. 5th edition. 2010 Philadelphia, PA, Mosby/Elsevier. Reproduced with permission.

Chapter 27

Algorithm 27.1 Diagnosis of chronic urticaria

Algorithm 27.2 Managment of chronic urticaria

Chapter 28

Algorithm 28.1 Diagnosis of hereditary angioedema

Figure 28.1 Extremity attack.

Figure 28.2 Abdominal swelling (arrow).

Figure 28.3 Airway angioedema.

Chapter 29

Algorithm 29.1 Diagnosis of anaphylaxis

Algorithm 29.2 Treatment of anaphylaxis

Chapter 30

Figure 30.1 Stroking one UP lesion has caused a linear raised area (wheal) with surrounding erythema to develop (Darier’s sign) on this child’s back. From Habif TP. Clinical Dermatology: A Color Guide to Diagnosis and Therapy. 5th edition. 2010 Philadelphia, PA, Mosby/Elsevier. Reproduced with permission.

Chapter 31

Algorithm 31.1 Diagnosis of insect sting allergy

Algorithm 31.2 Treatment of insect sting allergy

Chapter 33

Algorithm 33.1 Diagnosis of immediate allergic reactions to beta-lactams

Figure 33.1 The solid arrows indicate the core four-member beta-lactam ring within both penicillins and cephalosporins. Open arrows indicate the five- and six-member side rings for penicillins and cephalosporins, respectively. R indicates additional side chain sites where substitutions of various chemical groups produce different antimicrobial spectra, pharmacokinetics, or stability to beta-lactamases.

Chapter 34

Algorithm 34.1 Diagnosis of drug allergy

Chapter 36

Algorithm 36.1 Procedure of immunotherapy

Chapter 38

Algorithm 38.1 Evaluating the child with recurrent infections

Chapter 39

Algorithm 39.1 Evaluation of recurrent infections

Chapter 40

Figure 40.1 A 42-year-old healthy man developed pneumonia which led to an empyema. The culture grew

Streptococcus

pneumoniae

.

Chapter 41

Algorithm 41.1 Diagnosis of selective IgA deficiency

Algorithm 41.2 Treatment for selective IgA deficiency

Chapter 45

Algorithm 45.1 Diagnosis of chronic mucocutaneous candidiasis

Algorithm 45.2 Treatment of chronic mucocutaneous candidiasis

Chapter 46

Algorithm 46.1 Diagnosis of IPEX syndrome

Chapter 48

Algorithm 48.1 Diagnosis of hyper-IgE syndrome

Algorithm 48.2 Treatment of hyper-IgE syndrome

Chapter 49

Figure 49.1 Complement pathways and deficiencies. Deficiencies in complement increase susceptibility for infection by decreasing the ability for opsonization, which is particularly important for encapsulated bacteria. Additionally, a defect in the MAC decreases the ability of the immune system to generate lytic activity, which also increases the risk of infection with encapsulated bacteria.

Chapter 50

Figure 50.1 Phagocytic defect. A 16-month-old boy with no major past medical history developed fevers 2 weeks prior to admission. A biopsy shows granuloma and

Burkholderia cepacia

was cultured.

Chapter 51

Algorithm 51.1 Traditional HIV testing

Algorithm 51.2 New HIV testing

Algorithm 51.3 Management of the HIV positive child

Figure 51.1 Chest X-ray of an 18-year-old man with perinatally acquired HIV and

Pneumocystis

pneumonia showing bilateral reticulonodular densities. The patient’s CD4

+

lymphocyte count is 18/μL and the percentage CD4 lymphocyte count is 3%. At the time of presentation he had been non-adherent with trimethoprim-sulfamethoxazole which had been prescribed for

Pneumocystis

pneumonia prophylaxis.

Figure 51.2 Brain MRI image of an 18-year-old woman with perinatally acquired HIV and progressive multifocal leukoencephalopathy. She presented with left hemiparesis and a CD4

+

lymphocyte count of 8/μL. The MRI revealed T2 hyperintensities without contrast enhancement. A PCR for JC virus on the cerebrospinal fluid was positive.

Chapter 52

Algorithm 52.1 Diagnosis of HIVNote: EIA, enzyme immunoassay; NAAT, nucleic acid amplification test. If HIV RNA viral load is used as screening for acute HIV, a cutoff of 10 000 copies/mL is suggested because of the possibility of false positives with lower values.

Guide

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MOUNT SINAI EXPERT GUIDES

Allergy and Clinical Immunology

EDITED BY

Hugh A. Sampson, MD

Kurt Hirschhorn Professor of Pediatrics

Dean for Translational Biomedical Sciences

Director, Elliot and Roslyn Jaffe Food Allergy Institute

Division of Allergy/Immunology; Department of Pediatrics

Icahn School of Medicine at Mount Sinai

New York, NY, USA

This edition first published 2015 © 2015 by John Wiley & Sons, Ltd.

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Library of Congress Cataloging-in-Publication Data

Mount Sinai expert guides. Allergy & clinical immunology / edited by Hugh A. Sampson.  p. ; cm. Allergy & clinical immunology Includes bibliographical references and index.

 ISBN 978-1-118-60916-3 (pbk.)I. Sampson, Hugh A., editor. II. Title: Allergy & clinical immunology. [DNLM: 1. Hypersensitivity. 2. Allergens. 3. Immune System Diseases. 4. Immune System Phenomena. WD 300] RC584 616.97–dc23

      2014049390

A catalogue record for this book is available from the British Library.

Wiley also publishes its books in a variety of electronic formats. Some content that appears in print may not be available in electronic books.

Cover image: Stock Photo File #6124416 © David MarchalCover design by Ruth Bateson

Contributors

Shradha Agarwal MDAssistant Professor of MedicineDivision of Allergy/ImmunologyIcahn School of Medicine at Mount SinaiNew York, NY, USA

Supinda Bunyavanich MD, MPHAssistant ProfessorElliot and Roslyn Jaffe Food Allergy InstituteDivision of Allergy/ImmunologyDepartment of PediatricsDepartment of Genetics and Genomic SciencesIcahn School of Medicine at Mount SinaiNew York, NY, USA

Paula J. Busse MDAssociate ProfessorDepartment of MedicineDivision of Clinical ImmunologyIcahn School of Medicine at Mount SinaiNew York, NY, USA

Jean-Christoph Caubet MDChef de cliniqueDivision of AllergyDepartment of PediatricsUniversity Hospitals of GenevaGeneva, Switzerland

Mirna Chehade MD, MPHAssociate Professor of Pediatrics and MedicineElliot and Roslyn Jaffe Food Allergy InstituteDivision of Allergy/ImmunologyDepartment of PediatricsIcahn School of Medicine at Mount SinaiNew York, NY, USA

Beth E. Corn MDAssistant Professor of MedicineDepartment of Clinical ImmunologyIcahn School of Medicine at Mount SinaiNew York, NY, USA

Amanda L. Cox MDAssistant Professor of PediatricsElliot and Roslyn Jaffe Food Allergy InstituteDivision of Allergy/ImmunologyDepartment of PediatricsIcahn School of Medicine at Mount SinaiNew York, NY, USA

Charlotte Cunningham-Rundles MD, PhDProfessor of MedicineDepartments of Medicine and PediatricsThe David S. Gottesman ProfessorThe Immunology InstituteIcahn School of Medicine at Mount SinaiNew York, NY, USA

Elizabeth J. Feuille MDClinical FellowDivision of Allergy/ImmunologyIcahn School of Medicine at Mount SinaiNew York, NY, USA

Satish Govindaraj MDAssociate ProfessorDepartment of Otolaryngology–Head and Neck SurgeryIcahn School of Medicine at Mount SinaiNew York, NY, USA

Emma Guttman-Yassky MD, PhDAssociate Professor of Dermatology and ImmunologyDepartments of Dermatology and ImmunologyDirector, Center for Excellence in EczemaDirector, Occupational and Contact Dermatitis ClinicDirector, Laboratory for Investigation of Inflammatory Skin DiseasesIcahn School of Medicine at Mount SinaiNew York, NY, USA

Yan W. Ho MDResident of Otolaryngology ProgramDepartment of Otolaryngology–Head and Neck SurgeryIcahn School of Medicine at Mount SinaiNew York, NY, USA

Jacob D. Kattan MDAssistant Professor of Allergy and ImmunologyElliot and Roslyn Jaffe Food Allergy InstituteDivision of Allergy/ImmunologyDepartment of PediatricsIcahn School of Medicine at Mount SinaiNew York, NY, USA

Saakshi Khattri MDResearch AssociateLaboratory of Investigative DermatologyRockefeller UniversityNew York, NY, USA

Jennifer S. Kim MDAttending PhysicianNorthShore University HealthSystemEvanston, IL, USA;Elliot and Roslyn Jaffe Food Allergy InstituteDivision of Allergy/ImmunologyDepartment of PediatricsIcahn School of Medicine at Mount SinaiNew York, NY, USA

Michael Mullen MDProfessor of MedicineDirector, Institute of Advanced MedicineIcahn School of Medicine at Mount SinaiNew York, NY, USA

Anna Nowak-Węgrzyn MDAssociate Professor of PediatricsElliot and Roslyn Jaffe Food Allergy InstituteDivision of Allergy/ImmunologyDepartment of PediatricsIcahn School of Medicine at Mount SinaiNew York, NY, USA

Roberto Posada MDAssociate Professor of Pediatrics (Infectious Diseases) and Medical EducationDirector Pediatric, Adolescent and Young Adult HIV ProgramIcahn School of Medicine at Mount SinaiNew York, NY, USA

Elena S. Resnick MDAssistant Professor of Allergy and Clinical ImmunologyIcahn School of Medicine at Mount SinaiNew York, NY

Mariya RozenblitMedical StudentIcahn School of Medicine at Mount SinaiNew York, NY, USA

Hugh A. Sampson MDKurt Hirschhorn Professor of PediatricsDean for Translational Biomedical SciencesDirector, Elliot and Roslyn Jaffe Food Allergy InstituteDivision of Allergy/ImmunologyDepartment of PediatricsIcahn School of Medicine at Mount SinaiNew York, NY, USA

Gail F. Shust MDAssistant ProfessorDepartment of Pediatrics and Medical EducationIcahn School of Medicine at Mount SinaiNew York, NY, USA

Scott H. Sicherer MDElliot and Roslyn Jaffe Professor of Pediatrics, Allergy/ImmunologyJaffe Food Allergy InstituteIcahn School of Medicine at Mount SinaiNew York, NY, USA

Gwen S. Skloot MDAssociate Professor of MedicineDivision of Pulmonary,Critical Care and Sleep MedicineIcahn School of Medicine at Mount SinaiNew York, NY, USA

Timothy Sullivan MDInstructorDivision of Infectious DiseasesIcahn School of Medicine at Mount SinaiNew York, NY, USA

Julie Wang MDAssociate Professor of PediatricsElliot and Roslyn Jaffe Food Allergy InstituteDivision of Allergy/ImmunologyDepartment of PediatricsIcahn School of Medicine at Mount SinaiNew York, NY, USA

Kate Welch MDClinical FellowDivision of Allergy/ImmunologyIcahn School of Medicine at Mount SinaiNew York, NY, USA

Series Foreword

Now more than ever, immediacy in obtaining accurate and practical information is the coin of the realm in providing high quality patient care. The Mount Sinai Expert Guides series addresses this vital need by providing accurate, up-to-date guidance, written by experts in formats that are accessible in the patient care setting: websites, smartphone apps and portable books. The Icahn School of Medicine, which was chartered in 1963, embodies a deep tradition of pre-eminence in clinical care and scholarship that was first shaped by the founding of the Mount Sinai Hospital in 1855. Today, the Mount Sinai Health System, comprised of seven hospitals anchored by the Icahn School of Medicine, is one of the largest health care systems in the United States, and is revolutionizing medicine through its embracing of transformative technologies for clinical diagnosis and treatment. The Mount Sinai Expert Guides series builds upon both this historical renown and contemporary excellence. Leading experts across a range of disciplines provide practical yet sage advice in a digestible format that is ideal for trainees, mid-level providers and practicing physicians. Few medical centers in the USA could offer this type of breadth while relying exclusively on its own physicians, yet here no compromises were required in offering a truly unique series that is sure to become embedded within the key resources of busy providers. In producing this series, the editors and authors are fortunate to have an equally dynamic and forward-viewing partner in Wiley Blackwell, which together ensures that health care professionals will benefit from a unique, first-class effort that will advance the care of their patients.

Scott Friedman MDSeries EditorDean for Therapeutic DiscoveryFishberg Professor and Chief, Division of Liver DiseasesIcahn School of Medicine at Mount SinaiNew York, NY, USA

Preface

In the past 60 years, allergic disorders have increased dramatically in “westernized” countries, especially in North America, Europe and Australia/New Zealand. In the last half of the twentieth century, increases in respiratory allergies, i.e. asthma and allergic rhinitis, largely accounted for the rise in prevalence. However, in the last 15 years the prevalence of food allergies/anaphylaxis and atopic dermatitis have increased over two- to three-fold while the prevalence of respiratory allergies have leveled-off. Allergic disorders now affect about one-third of the US population and cost the health care system several billions of dollars each year in medical expenses and lost wages. Drug allergy is estimated to affect about 10% of the world’s population and 20% of hospitalized patients. Over the past two decades, scientists and clinicians have made great strides in the diagnosis and management of allergic disorders. The past several decades have also witnessed tremendous advances in our recognition of immunodeficiency disorders and our understanding of the human immune system. Advances in molecular genetic techniques and other technical advances have led to the identification and characterization of many new immunodeficiency disorders that have enabled clinicians to more appropriately treat patients afflicted with these disorders. Early recognition and initiation of therapy is key to preserving a more normal life for children afflicted with these disorders. In addition, investigators have now recognized that adults also develop various forms of primary and secondary immunodeficiency diseases and require timely evaluation and management of their disorder.

In this book, members of the pediatric and adult Divisions of Allergy and Immunology at the Icahn School of Medicine at Mount Sinai have teamed-up to provide a clinician-friendly manual outlining the diagnosis and management of a wide variety of allergic and immunodeficient disorders. Members of the Allergy/Immunology group have provided a brief outlines on the etiology and pathogenesis of various disorders, succinct guidelines on the relevant historical and laboratory information necessary for establishing a timely and accurate diagnosis, guidelines on the most current forms of therapy to manage the various disorders, and a brief discussion of anticipated natural history and outcomes. Each chapter author provides her/his perspective on evaluating and managing various allergic and immunologic disorders based on years of experience dealing with allergic and immunodeficient patients. This book is not meant to be an exhaustive discussion of allergic/immunologic conditions (although references are provided to satisfy the most inquisitive reader), but a useful guidebook enabling the busy clinician to recognize patients afflicted with allergic or immunologic disorders and provide them with the most current management strategies.

Hugh A. Sampson, MDKurt Hirschhorn Professor of PediatricsDean for Translational Biomedical SciencesDirector, Elliot and Roslyn Jaffe Food Allergy InstituteDivision of Allergy/Immunology; Department of PediatricsIcahn School of Medicine at Mount SinaiNew York, NY, USA

List of Abbreviations

ABG

arterial blood gas

ACD

allergic contact dermatitis

ACE-I

angiotensin-converting enzyme inhibitor

AD

atopic dermatitis

ADA

adenosine deaminase

AD-HIES

autosomal dominant hyper IgE syndrome

AGEP

acute generalized exanthematous pustulosis

AHR

airway hyper-responsiveness

AKC

atopic keratoconjunctivitis

ALPS

autoimmune lymphoproliferative syndrome

APECED

autoimmune polyendocrinopathy, candidiasis, and ectodermal dystrophy (syndrome)

APT

atopy patch test

ASM

aggressive systemic mastocytosis

BMP

basic metabolic panel

BP

blood pressure

cART

combination antiretroviral therapy

CBC

complete blood count

CD

celiac disease

CDC

Centers for Disease Control and Prevention

CGD

chronic granulomatous disease

CLL

chronic lymphocytic leukemia

CM

cow’s milk

CM

cutaneous mastocytosis

CMC

chronic mucocutaneous candidiasis

CMV

cytomegalovirus

COPD

chronic obstructive pulmonary disease

CRP

C-reactive protein

CSF

cerebrospinal fluid

CT

computed tomography

CU

chronic urticaria

CVID

common variable immunodeficiency

DAF

decay accelerating factor

DBPCFC

double-blind, placebo-controlled oral food challenge

DCM

diffuse cutaneous mastocytosis

DGP

deamidated gliadin peptide

DLCO

diffusion capacity for carbon monoxide

DRESS

drug reaction or rash with eosinophilia and systemic symptom

EASI

Eczema Area and Severity Index

EG

eosinophilic gastroenteritis

EGD

esophagogastroduodenoscopy

EIA

enzyme immunoassay

EoE

eosinophilic esophagitis

ESR

erythrocyte sedimentation rate

FDA

Food and Drug Administration

FDEIA

food-dependent, exercised-induced anaphylaxis

FeNO

fractional exhaled nitric oxide

FESS

functional endoscopic sinus surgery

FEV

1

forced expiratory volume in 1 second

FISH

fluoroescence in situ hybridization

FPIES

food protein-induced enterocolitis syndrome

FPIP

food protein-induced proctocolitis

FVC

forced vital capacity

G-CSF

granulocyte colony-stimulating factor

GERD

gastroesophageal reflux disease

GI

gastrointestinal

GM-CSF

granulocyte macrophage colony-stimulating factor

GPC

giant papillary conjunctivitis

HAE

hereditary angioedema

H&E

hematoxylin and eosin

HEENT

head, eyes, ears, nose, and throat

HEPA

high-efficiency particulate air

HES

hypereosinophilic syndrome

5-HIAA

5-hydroxyindoleacetic acid

HIES

hyper IgE syndrome

HLA

human leukocyte antigen

HMW

high molecular weight

HPF

high power field

HSCT

hematopoietic stem cell transplantation

HUS

hemolytic uremic syndrome

HVAC

heating, ventilating, and air conditioning

IFA

immunofluorescent antibody

IFN

interferon

Ig

immunoglobulin

IL

interleukin

IPEX

immune dysregulation, polyendocrinopathy, X-linked

ISM

indolent systemic mastocytosis

ITP

immune thrombocytopenia

IVIg

intravenous immunoglobulin

LAD

leukocyte adhesion deficiency

LFT

liver function test

LMW

low molecular weight

LTC4

leukotriene C4

LTP

lipid transfer protein

MAC

membrane attack complex

MASP

mannan-binding lectin-associated protease

MBL

mannose-binding lectin

MBP

mannose-binding protein

MCAS

mast cell activation syndrome

MCL

mast cell leukemia

MHC

major histocompatibility complex

MMAS

monoclonal mast cell activation syndrome

MRI

magnetic resonance imaging

MRSA

methicillin-resistant

Staphylococcus aureus

MSG

monosodium glutamate

NARES

non-allergic rhinitis with eosinophilia

NIH

National Institutes of Health

NRTI

nucleoside reverse-transcriptase inhibitor

NSAID

non-steroidal anti-inflammatory drug

OA

occupational asthma

OAS

oral allergy syndrome

OFC

oral food challenge

OMC

osteomeatal complex

PAC

perennial allergic conjunctivitis

pd

plasma-derived

PE

pulmonary embolism

PEFR

peak expiratory flow rate

PEP

post-exposure prophylaxis

PGD2

prostaglandin D2

PLE

protein-losing enteropathy

PMTCT

prevention of mother-to-child transmission

PNH

paroxysmal nocturnal hemoglobinuria

ppb

parts per billion

PPI

proton pump inhibitor

PrEP

pre-exposure prophylaxis

RADS

reactive airway dysfunction syndrome

RAST

radioallergosorbent test

RSV

respiratory syncytial virus

SAC

seasonal allergic conjunctivitis

SCF

stem cell factor

SCID

severe combined immunodeficiency

SCIT

subcutaneous immunotherapy

SCORAD

Severity Scoring of Atopic Dermatitis

SJS

Stevens–Johnson syndrome

SLE

systemic lupus erythematosus

SLIT

sublingual immunotherapy

SM

systemic mastocytosis

SM-AHNMD

systemic mastocytosis with an associated hematologic non-mast cell lineage disorder

SNOT

sinonasal outcome test

TEN

toxic epidermal necrolysis

TMEP

telangiectasia macularis eruptiva perstans

TNF

tumor necrosis factor

TREC

T-cell receptor excision circle

Treg

regulatory T-cell

TSH

thyroid stimulating hormone

TTG

tissue transglutaminase

UACS

upper airway cough syndrome

UP

urticaria pigmentosa

URI

upper respiratory infection

URTI

upper respiratory tract infection

VIP

vasoactive intestinal peptide

VIT

venom immunotherapy

VKC

vernal keratoconjunctivitis

VL

viral load

WAS

Wiskott–Aldrich syndrome

WASp

Wiskott–Aldrich syndrome protein

WB

Western blot

WHIM

warts, hypogammaglobulinemia, infections, and myelokathexis (syndrome)

WHO

World Health Organization

WIP

WASp-interacting protein

XLN

X-linked neutropenia

XLT

X-linked thrombocytopenia

About the Companion Website

This series is accompanied by a companion website:

www.mountsinaiexpertguides.com

The website includes:

Case studies

ICD codes

Interactive MCQs

Patient advice

PART 1Allergy

CHAPTER 1Atopic Dermatitis in Infants and Young Children

Jean-Christoph Caubet1 and Anna Nowak-Węgrzyn2

1Division of Allergy, Department of Pediatrics, University Hospitals of Geneva, Geneva, Switzerland

2Department of Pediatrics, Division of Allergy/Immunology, Jaffe Food Allergy Institute, Icahn School of Medicine at Mount Sinai, New York, NY, USA

OVERALL BOTTOM LINE

Atopic dermatitis (AD) is a common, chronic, relapsing, inflammatory skin disorder and may be the initial step of the so-called atopic march.

Pathogenesis is complex and not fully understood, but recent investigations have highlighted two cornerstone features of AD: defective epidermal barrier and cutaneous inflammation involving both IgE- and T-cell-mediated responses.

Diagnosis of AD is based on clinical features.

A complete allergy investigation is required in patients with moderate to severe AD or a history of exacerbation after food ingestion. The younger the age of onset and the more severe the rash, the more likely foods are to trigger AD in children.

Although there is no cure for AD, the goals of treatment are to reduce symptoms, prevent exacerbations, minimize side effects, and provide adequate psychological support.

Section 1: Background

Definition of disease

AD is a familial, common, inflammatory skin disorder characterized by chronically relapsing course and intensely pruritic eczematous flares. The term “eczema” alone generally refers to AD and these terms are often used interchangeably.

Disease classification

Although clinically indistinguishable, AD has been categorized into an immunoglobulin E (IgE) associated form (true AD, formerly called extrinsic AD) and a non-IgE-associated form (“non-atopic” dermatitis, formerly called intrinsic AD). However, this classification is controversial as the absence of sensitization to common food allergens and aeroallergens may be only a transient factor.

Incidence/prevalence

AD affects an estimated 18 million people in the United States.

The estimated lifetime prevalence in children ranges from 10–30%.

Wide variations in prevalence have been observed between countries, suggesting that environmental factors determine AD expression.

Economic impact

The economic impact of AD is important and will likely increase in proportion to increasing disease prevalence. The current estimates range widely, from $364 million to $3.8 billion dollars per year.

Etiology

The exact etiologic factors leading to AD are not well defined.

Genetically predisposed patients with AD have an epidermal barrier dysfunction, linked to decreased or impaired function of essential barrier proteins (i.e. filaggrin and ceramide).

Common triggers in AD include food proteins, aeroallergens, stress, climate, irritants, and microbes.

Pathology/pathogenesis

AD is mainly characterized by a defective epidermal barrier and cutaneous inflammation.

Genetically predisposed patients with AD have an epidermal barrier dysfunction; contributing factors include decreased ceramide levels and loss of function of crucial protein (e.g. filaggrin). This can result in enhanced transepidermal water loss and facilitated penetration of environmental allergens, promoting allergic skin inflammation.

The complex underlying immune response of AD involves both IgE-mediated and T-cell-mediated delayed immune responses. Acute skin lesions of AD are characterized by cells containing Th2 cytokines (i.e. IL-4, IL-5, IL-13 and IL-22), whereas Th1 cells expressing γ-interferon (IFN-γ) are most commonly found in more chronic lesions.

Although elevated serum total IgE levels can be found in 80–85% of patients with AD, the clinical relevance of associated sensitizations has been difficult to ascertain.

Foods can cause exacerbation in a subset of patients (i.e. approximately one-third of young children with a moderate or severe AD). Similarly, exacerbation of AD can occur with exposure to aeroallergens such as house dust mites, animal danders, and pollens.

Most patients with AD have an inadequate innate immune response to epicutaneous microbes, in part responsible for increased susceptibility to infections (bacteria, yeast, viruses) and colonization with

Staphylococcus aureus.

These microbes contribute, at least partially, to the skin inflammation and can potentially lead to exacerbations of the disease.

Predictive/risk factors

Risk factor

Odds ratio

Genetic factors (filaggrin mutation)

3.73–7.1

Breastfeeding

Controversial

Tobacco

1.97

Familial history of atopy

2–6

Section 2: Prevention

BOTTOM LINE/CLINICAL PEARL

For infants at high risk of atopy, exclusive breastfeeding for at least 4 months and/or feeding with extensively hydrolyzed formula decreases cumulative incidence of AD in the first 2 years of life. There is no prevention strategy proven to protect beyond the first few years of life.

Screening

Not applicable for this topic. Studies on screening for filaggrin mutations are ongoing.

Primary prevention

Exclusive breastfeeding for at least 4 months and/or feeding with extensively hydrolyzed formula decreases cumulative incidence of AD in the first 2 years of life.

Although several studies support a preventative effect of treating with probiotics during pregnancy or early infancy to delay the onset of AD, controversy persists and more studies are needed to confirm these data.

Secondary prevention

Optimal skin care remains the cornerstone of the management of AD.

Avoidance of common irritants and specific allergen triggers (foods and/or aeroallergens) in selected patients constitute a large part of secondary prevention of AD.

Other important measures include control of household temperature and humidity; use of mild soaps for bathing (neutral pH and minimal defatting capabilities); bathing in warm water once a day for 15–20 minutes, pat dry and immediate application of emollients; nails trimming to decrease abrasion to skin; use of clothing made of cotton instead of synthetic fibers and wool.

Section 3: Diagnosis (Algorithm 1.1)

Algorithm 1.1Diagnosis of atopic dermatitis

BOTTOM LINE/CLINICAL PEARLS

The diagnosis of AD is based on a constellation of clinical features.

Recurrent pruritus is the only symptom of AD.

Physical examination reveals xerosis and typical eczematous lesions with different morphologic aspects and locations depending on the age of the patient. Eczematous rashes tend to be generalized and affect the face and extensor surfaces of the limbs in infants and toddlers, while in older children and adults rashes localize to the peri-orbital area, flexor surfaces of the joints, and about the wrists and ankles.

Allergic investigations are usually not indicated for patients with mild AD.

In patients with moderate–severe atopic dermatitis or with a positive history of exacerbation after exposure to a specific allergen, an allergy investigation including skin tests, specific IgE measurement, and/or an oral provocation test are indicated.

Differential diagnosis

Differential diagnosis

Features

Scabies

Papules, finger web involvement, positive scraping for scabies mite

Allergic contact dermatitis

Positive exposure history, rash in area of exposure, absence of family history

Seborrheic dermatitis

Greasy, scaly lesions, absence of family history

Zinc and biotin deficiency

Eczematous lesions localized in peri-oral area and rectum (oral, anal)

Psoriasis

Localized patches on extensor surfaces, scalp, buttocks, pitted nails

Ichthyosis

Usually non-pruritic, not the typical distribution pattern seen in AD, no inflammatory lesions (except in Netherton’s syndrome)

Netherton’s syndrome (severe, autosomal recessive form of ichthyosis associated with mutations in the

SPINK5

gene)

Chronic skin inflammation, universal pruritus, severe dehydration and stunted growth, hair shaft defect (trichorrhexis invaginata), also known as “bamboo hair”

Immunodeficiency: severe combined immunodeficiency syndrome, hyper-IgE syndrome, Wiskott–Aldrich syndrome

Severe eczema, positive history of multiple infections, growth failure

Typical presentation

Atopic dermatitis occurs in the first year of life in 60% of cases, and by the age of 5 years in nearly 85% of cases. Patients typically present with pruritus and chronically relapsing/remitting eczematous lesions having a typical morphology and distribution related to the age of the patient.

Clinical diagnosis

History

The diagnosis of AD is based on a constellation of clinical features, pruritus being the cardinal symptom of this disorder.

One of the major clinical features of AD is its chronicity, characterized by an intermittent course with flares and remission.

A careful allergy history is of major importance to identify potential allergen triggers (e.g. aeroallergens and foods), particularly in patients with moderate–severe AD. Identification of common irritants is also an important part of the history.

The clinician should evaluate the impact on quality of life, particularly sleeping and psychologic aspects.

Physical examination

Characteristic skin findings in AD include primarily xerosis and eczematous lesions with different morphologic aspects and locations depending on the age of the patient.

Acute and subacute eczematous skin lesions are typically found in infants and young children, with intensely pruritic, erythematous, papulo-vesicular lesions, excoriation, and serous exudate. The lesions are typically localized on the scalp, face (cheeks and chin), and extensor surfaces of the extremities. In older children, lesions are more commonly found in the flexor surfaces (antecubital and popliteal fossa), neck, wrists, and ankles.

Lichenification is rarely seen in infancy but is characteristic of childhood AD.

Of note, peri-orbital hyperpigmentation and Dennie–Morgan folds (prominent folds of skin under the lower eyelid) are common peri-ocular findings in patients with AD.

Useful clinical decision rules and calculators

The UK diagnostic criteria are the most extensively validated for AD and are based on the classic diagnostic criteria of Hanifin and Rajka.

The patient must have an itchy skin condition in the last 12 months plus three or more of the following criteria:

Onset below age 2 (not used in children under 4 years);

History of flexural involvement;

History of a generally dry skin;

Personal history of other atopic disease (in children aged under 4 years, history of atopic disease in a first degree relative may be included); and

Visible flexural dermatitis as per photographic protocol.

Disease severity classification

There are many tools to evaluate the severity of AD, although they have been used mainly in clinical research trials. The most well known:

Severity Scoring of Atopic Dermatitis (SCORAD):

uses the “rule of 9’s” to assess disease extent and evaluates five clinical characteristics to determine disease severity; and

Eczema Area and Severity Index (EASI):

assesses extent of diseases at four body sites and measures four clinical signs on a scale of 0–3.

Laboratory diagnosis

List of diagnostic tests

Skin prick tests and/or specific IgE to common food allergens should be restricted to patients with moderate–severe AD or to patients having a positive history of exacerbation after specific food ingestion.

The most common food allergens in childhood AD are hen’s egg, cow’s milk, peanut, soybean, wheat, tree nuts, fish, and shellfish. Hen’s egg, cow’s milk, and peanut account for about 80% of food allergy diagnosed by food challenge in children with AD.

Skin prick tests and/or specific IgE to aeroallergens (i.e. pollens, animal danders, and dust mites) should be performed according to the history and the age of the patient.

Atopy patch testing is an additional tool in selected cases in which skin prick tests or specific IgE fail to identify a suspected food. It is not recommended as a routine diagnostic test in AD.

Oral food challenges are considered the gold standard to diagnose an associated food allergy.

Scraping to exclude tinea corporis is occasionally helpful.

A swab of infected skin may help with the isolation of a specific organism and antibiotic sensitivity assessment.

Skin biopsy is usually not required to confirm the diagnosis of AD but in rare difficult cases it can be useful to exclude other causes.

Lists of imaging techniques

Not applicable for this topic.

Potential pitfalls/common errors made regarding diagnosis of disease