Interdisciplinary Approaches to Overlap Disorders in Dermatology & Rheumatology

Foreword
Preface
Contents
Contributors
1: Evaluation of the Integumentary and Musculokeletal Systems: An Approach to the Interdisciplinary Examination for Overlap Diseases
	Introduction
	General Recommendations
	Distribution
	Color
	Shape or Configuration
	Scaling
	Consistency
	Scalp/Hair/Nails/Oral Mucosa
	Rheumatologic Musculoskeletal Examination
	Joint Examination
	Muscle Examination
	Conclusion
2: Serologic Evaluation in the Rheumatology-Dermatology Overlap Patient
	Interdisciplinary Introduction
	Erythrocyte Sedimentation Rate and C-reactive Protein
	Antibodies in Rheumatologic Disease – General Principles
	Anti-nuclear Antibody
	SLE-Associated Serologies
	Systemic Sclerosis (Scleroderma)
	Dermatomyositis
	Psoriatic Arthritis
	Vasculitis
	Still’s Disease
	Sarcoidosis
	Lyme disease
	Summary
	Suggested Reading
		References
3: Cutaneous Lupus
	Interdisciplinary Introduction
	Epidemiology & Classification
		Epidemiology
		Classification of Cutaneous Lupus Erythematosus
			Gilliam Classification (Table 3.1)
			Other Approaches to CLE Subgrouping
			LE-Nonspecific Skin Changes (Table 3.2)
	CLE Association with SLE
	Pathogenesis of Cutaneous Lupus Erythematosus
		Genetic Factors
		Ultraviolet Light and Apoptosis
		Innate Immunity
		Inflammatory Cells
		Inflammatory Cytokines and Chemokines
	Clinical Features
	Acute Cutaneous Lupus Erythematosus (ACLE)
	Subacute Cutaneous Lupus Erythematosus (SCLE)
	Discoid Lupus Erythematosus (DLE)
	Cutaneous Lupus: Additional LE-Specific Skin Variants
		Lupus Erythematosus Tumidus (Tumid Lupus)
		Lupus Erythematosus Panniculitis/Lupus Profundus
		Chilblain Lupus
		Neonatal Lupus
	Lupus-specific Vesiculobullous Disease
		Bullous SLE (BSLE)
		Vesiculobullous Disease Occurring as Severe Variants of ACLE, SCLE, and Rarely, DLE.
	Non-specific Cutaneous Lesions of LE
	Diagnostic Considerations
		Diagnosis of Cutaneous Lupus
			Histopathology
			Antibody Deposits in the Skin (DIF)
	Autoantibodies
	Evaluation for Systemic Disease
	Principles of Management
		Prevention/Patient Education
			Sun Protection
			Smoking Cessation
			Camouflage
		Local Therapy
			Topical Steroids
			Calcineurin Inhibitors
		Intralesional corticosteroids
			Laser
		Systemic Therapy
			Antimalarials
			Antimalarial-resistant Disease
			Immunosuppressant Agents
				Methotrexate
				Mycophenolate Mofetil (MMF) or Mycophenolate Sodium
			Azathioprine
		Systemic Corticosteroids
		Immunomodulators
			Dapsone
			Thalidomide
			Lenalidomide
			Oral Retinoids
		Other Therapies
	Disease and Comorbidity Assessment (Table 3.6)
		Systemic Screening
		Malignancy Risk
		Pregnancy
	Summary
	References
4: Dermatomyositis
	Interdisciplinary Introduction
	Epidemiology and Risk Factors
	Classification
	Clinical Presentation
		Skin Disease
			Classic Features
			Rare Presentations of Cutaneous DM
			Cutaneous Signs of Interstitial Lung Disease
			Cutaneous Signs of Internal Malignancy
			Histopathology
			Differential Diagnosis
		Muscle Disease
			Classic Presentation
			Histopathology
			Electromyography
			Imaging
			Differential Diagnosis
		Systemic Disease
			Pulmonary Manifestations
				Interstitial Lung Disease
				Pulmonary Arterial Hypertension
			Cardiac Manifestations
				DM-Specific Cardiac Involvement
				Coronary Artery Disease
			Thromboembolic Disease
			Joint Manifestations
			Renal Manifestations
			Rare Manifestations
			Cancer
		Principles of Management
			Overview
			Behavioral Change
			Physical Medicine and Rehabilitation
			Topical Agents
				Topical Corticosteroids
				Topical Calcineurin Inhibitors
			Systemic Medications
				Antimalarials
				Methotrexate
				Systemic Corticosteroids
				Mycophenolate Mofetil
				Intravenous Immunoglobulin
				Rituximab
				Cyclosporine
				Tofacitinib
				Tacrolimus
				Nonsteroidal Anti-inflammatory Drugs
				Azathioprine
				Leflunomide
				Dapsone
				Thalidomide
				Cyclophosphamide
			Special Cases: Calcinosis Cutis and Ulceration
			Summary
	References
5: Morphea
	Interdisciplinary Introduction
	Nomenclature
	Epidemiology
	Pathogenesis
		Evidence for Autoimmunity
		Genetic Susceptibility
		Environmental Triggers
	Histopathology
	Classification
		Peterson Criteria (1995)
		Laxer and Zulian (2006)
	Clinical Features
		Plaque Morphea
		Generalized Morphea
		Linear Morphea
		Deep Morphea
			Disabling Pansclerotic Morphea
			Eosinophilic Fasciitis
		Mixed Morphea
		Rare Variants
	Natural History
	Disease Assessment
	Comorbidities
	Approach to Screening and Monitoring
	Principals of Management
		Topical Therapy
		Phototherapy
		Systemic Therapy
	Summary
	References
6: Systemic Sclerosis
	Interdisciplinary Introduction
	Nomenclature
	Epidemiology
	SSc Classification Criteria
	SSc Subsets
	Clinical Features
		Cutaneous
		Vascular
			Raynaud’s Phenomenon
			Digital Ischemia
		Gastrointestinal
			Oropharynx
			Esophagus
			Gastric
			Small Intestine
			Large Intestine and Anorectum
			Liver
		Pulmonary
			Interstitial Lung Disease
			Pulmonary Hypertension
		Cardiac
		Renal
		Musculoskeletal
	Pathophysiology of Systemic Sclerosis
		Vascular Injury and the Initiation of SSc
		Autoimmunity
		Fibrosis
	Risk Factors for Systemic Sclerosis
		Genetic Risk Factors
			Occupational and Environmental Risk Factors
	Autoantibodies
	Diagnostic Considerations
		Physical Examination
		Laboratory Testing
		Cardiopulmonary Studies
		Other Studies
	Disease and Comorbidity Assessment
		Measurement of Disease Activity and Severity
		Monitoring
		Comorbidities
	Management of SSc
		Organ-Specific Therapy
			Skin Disease
			Vascular: Raynaud’s Phenomenon and Digital Ischemic Ulcers
			Gastrointestinal
			Pulmonary
				Interstitial Lung Disease
				Pulmonary Arterial Hypertension
			Renal
			Musculoskeletal
		Immune Modulation and Targeted Therapies
	Survival
	Summary
	References
7: Psoriasis and Psoriatic Arthritis
	Interdisciplinary Introduction
		Epidemiology & Classification
		Pathogenesis
		Clinical Features
		Diagnostic Considerations
	Screening Tools
		Disease and Comorbidity Assessment
			Gastrointestinal Disease
			Autoimmune Ophthalmic Disease
			Psychiatric Disease
			Metabolic Syndrome and Cardiovascular Disease
		Principles in Management
			Disease Outcome Measures
	Minimal Disease Activity State and Treat to Target
		Therapeutic Interventions
			Lifestyle Modifications
			Topical Therapy for Psoriasis
			Phototherapy
			Oral Therapy
				Non-steroidal Anti-inflammatory Drugs and Corticosteroids
				Methotrexate
				Leflunomide
				Sulfasalazine
				Acitretin
				Cyclosporine
				Apremilast
	Biologic Therapy
		TNF-α Antagonists
		Etanercept
		Adalimumab
		Infliximab
		Golimumab
		Certolizumab
		Risks and Benefits of Using TNF-α Antagonists
		IL-12 / IL-23 Antagonists
			Ustekinumab
			Guselkumab
			Risankizumab and Tildrakizumab
			Safety Concerns with IL-12/23 Antagonists
		IL-17 Antagonists
			Secukinumab
			Ixekizumab
			Brodalumab
			Safety Concerns with IL-17 Antagonists
		Janus Kinase Inhibitors
			Safety Concerns with JAK Inhibitors
		T Cell Costimulation Blockade
			Safety Concerns with T Cell Co-stimulation Blockade
			Monitoring in Biologics
			Combining Psoriasis Therapies
			Cost of Psoriasis and Psoriatic Arthritis Therapies
	References
8: Vasculitis
	Interdisciplinary Introduction
	Nomenclature and Classification of Vasculitis
	Pathophysiology and Clinical Features
		Small-Vessel Vasculitis
		Medium-Vessel Vasculitis
		Extracutaneous Manifestations
	Types of Vasculitis with Skin Involvement
		Cutaneous Small-Vessel Vasculitis/Small-Vessel Vasculitis of the Skin
			Cutaneous Manifestations
			Systemic Findings
		IgA Vasculitis
			Cutaneous Manifestations
			Systemic Findings
		Urticarial Vasculitis
			Cutaneous Manifestations
			Systemic Findings
		Cryoglobulinemic Vasculitis
			Cutaneous Manifestations
			Systemic Findings
		Granulomatosis with Polyangiitis (Wegner’s)
			Cutaneous Manifestations
			Systemic Findings
		Microscopic Polyangiitis
			Cutaneous Manifestations
			Systemic Findings
		Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss)
			Cutaneous Manifestations
			Systemic Findings
		Polyarteritis Nodosa
			Cutaneous Manifestations
			Systemic Findings
		Giant Cell Arteritis and Takayasu Arteritis
	Initial Evaluation of Patients with Possible Vasculitis
		Skin Biopsy
			Biopsy Selection and Performance
			Histologic Findings in Cutaneous Vasculitis
			Common Pitfalls in Using Skin Pathology to Evaluate Possible Vasculitis
		Review of Systems and Medical History
		Laboratory Studies
			Basic Tests That Should Be Ordered in All Patients
	Urinalysis with Microscopic Examination
	Basic Metabolic Panel
	Complete Blood Count
	Other Laboratory Tests Useful in Select Cases
		Liver Function Tests
		Acute Phase Reactants
		Serologic Tests of Autoimmunity
		Antineutrophilic Cytoplasmic Antibodies (ANCAs)
		Anti-Nuclear Antibodies
		Rheumatoid Factor
		Complement Levels
		Cryoglobulins
		Serum Protein Electrophoresis (SPEP) and Immunofixation
		Serologic Tests for Infections
		Urine Drug Screen
		Diagnostic Imaging Studies in the Evaluation of Possible Vasculitis
			Chest Imaging
			Sinus and Upper Airway Imaging
			Catheter-Based Angiography, MR Angiography, and CT Angiography
			Nerve Conduction Studies and Electromyography
	Workup and Treatment of Specific Vasculitides with Skin Involvement
		Cutaneous Small-Vessel Vasculitis/Small-Vessel Vasculitis of the Skin
			Diagnostic Evaluation
			Treatment
		IgA Vasculitis
			Diagnostic Evaluation
			Treatment
		Urticarial Vasculitis
			Diagnostic Evaluation
			Treatment
		Cryoglobulinemic Vasculitis
			Diagnostic Evaluation
			Treatment
		Granulomatosis with Polyangiitis (Wegener’s)
			Diagnostic Evaluation
			Treatment
		Microscopic Polyangiitis
			Diagnostic Evaluation
			Treatment
		Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss)
			Diagnostic Evaluation
			Treatment
		Polyarteritis Nodosa
			Diagnostic Evaluation
			Treatment
	Referral and Coordination of Care
	Summary
	References
9: Sarcoidosis
	Interdisciplinary Introduction
	Epidemiology & Risk Factors
	Pathogenesis
	Clinical Features
		Pulmonary Sarcoidosis
		Cutaneous Sarcoidosis
		Other Organ Involvement
		Specific Sarcoidosis Phenotypes
	Diagnostic Considerations
	Disease and Comorbidity Assessment
	Principles of Management
		Treatment for Cutaneous Sarcoidosis
		Treatment of Pulmonary Sarcoidosis
	Summary
	References
10: Reactive Erythemas and Panniculitides in Connective Tissue Disease
	Interdisciplinary Introduction
	Erythema Nodosum, Erythema Induratum, and Connective Tissue Panniculitides
	Erythema Nodosum
		Key Summary Capsule Bullets
		Classification and Epidemiology
		Pathogenesis
		Clinical Features
		Histopathology
		Diagnostic Considerations
		Disease and Comorbidity Assessment
		Principles of Management
	Erythema Induratum/Nodular Vasculitis
		Key Summary Capsule Bullets
		Classification
		Clinical Features
		Principles of Management
	Lupus Erythematosus Panniculitis
		Key Summary Capsule Bullets
		Classification and Epidemiology
		Pathogenesis
		Clinical Features
		Diagnostic Considerations
		Disease and Comorbidity Assessment
		Principles of Management
	Dermatomyositis-Associated Panniculitis
		Key Summary Capsule Bullets
		Classification and Epidemiology
		Pathogenesis
		Clinical Features
		Diagnostic Considerations
		Disease and Comorbidity Assessment
		Principles of Management
	Panniculitis of Sclerosing Disorders
		Key Summary Capsule Bullets
		Classification
		Clinical Features
		Principles of Management
	Pyoderma Gangrenosum
		Key Summary Capsule Bullets
		Classification and Epidemiology
		Pathogenesis
		Clinical Features
		Diagnostic Considerations
		Disease and Comorbidity Assessment
		Principles of Management
	Sweet Syndrome
		Key Summary Capsule Bullets
		Classification and Epidemiology
		Pathogenesis
		Clinical Features
		Diagnostic Considerations
		Disease and Comorbidity Assessment
		Principles of Management
	Palisaded Neutrophilic Granulomatous Dermatitis and Interstitial Granulomatous Dermatitis
	Palisaded Neutrophilic Granulomatous Dermatitis
		Key Summary Capsule Bullets
		Classification and Epidemiology
		Pathogenesis
		Clinical Features
		Diagnostic Considerations
		Disease and Comorbidity Assessment
		Principles of Management
	Interstitial Granulomatous Dermatitis and Interstitial Granulomatous Drug Reaction
		Key Summary Capsule Bullets
		Classification and Epidemiology
		Pathogenesis
		Clinical Features
			Interstitial Granulomatous Dermatitis
			Interstitial Granulomatous Drug Reaction
		Diagnostic Considerations
		Disease and Comorbidity Assessment
		Principles of Management
	Summary
	References
11: Iatrogenic Disease and Drug Induced Toxicities Related to Anti-Inflammatory and Immunomodulatory Agents
	Interdisciplinary Introduction
	Toxicities of Anti-Inflammatory and Immunomodulatory Agents
		Antimalarials
			Mechanism of Action
			Cutaneous Side Effects
			Extracutaneous and Systemic Side Effects
		Azathioprine
			Mechanism of Action
			Cutaneous Side Effects
			Extracutaneous and Systemic Side Effects
		Cyclosporine
			Mechanism of Action
			Cutaneous Side Effects
			Extracutaneous and Systemic Side Effects
		Dapsone
			Mechanism of Action
			Cutaneous Side Effects
			Extracutaneous and Systemic Side Effects
		Mycophenolate Mofetil
			Mechanism of Action
			Cutaneous Side Effects
			Extracutaneous and Systemic Side Effects
		Methotrexate
			Mechanism of Action
			Cutaneous Side Effects
			Extracutaneous and Systemic Side Effects
		Tumor Necrosis Factor Inhibitors
			Mechanism of Action
			Cutaneous Side Effects
			Extracutaneous and Systemic Side Effects
	Drug-Induced Lupus Erythematosus and Drug-Induced Dermatomyositis
		Drug-Induced Lupus Erythematosus
		Drug-Induced Dermatomyositis
		Summary
	References
Index