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Dermatology
Other Rheumatologic Disorders and Autoinflammatory Diseases
Built from Dermatology, 5th Edition
What’s inside
8 sections · 129 slides
Overview
- What this topic covers
- Innate versus adaptive: two disease patterns
Systemic-Onset Juvenile Idiopathic Arthritis
Still disease in children
- What is systemic-onset juvenile idiopathic arthritis
- Classification of juvenile idiopathic arthritis
- Classification of juvenile idiopathic arthritis
- Classification of juvenile idiopathic arthritis (continued)
- Epidemiology of systemic-onset JIA
- Pathogenesis: an innate immune, not autoimmune, disease
- How IL-1 and IL-6 cause the features of sJIA
- Fever and rash pattern in sJIA
- Evanescent rash and persistent plaques in systemic-onset JIA
- Linear array of evanescent papules and plaques in a child
- Joints, labs, and the macrophage activation syndrome
- Skin biopsy findings in sJIA
- Differential diagnosis of fever, rash and joint pain
- Hereditary periodic fever syndromes
- Hereditary periodic fever syndromes
- Hereditary periodic fever syndromes (continued)
- Hereditary periodic fever syndromes (continued)
- Hereditary periodic fever syndromes (continued)
- Hereditary periodic fever syndromes (continued)
- Hereditary periodic fever syndromes (continued)
- Hereditary periodic fever syndromes (continued) (continued)
- Hereditary periodic fever syndromes (continued) (continued)
- Hereditary periodic fever syndromes (continued) (continued)
- Hereditary periodic fever syndromes (continued) (continued)
- Hereditary periodic fever syndromes (continued)
- Hereditary periodic fever syndromes (continued) (continued)
- Treatment of systemic-onset JIA
Adult-Onset Still Disease
Still disease that begins in adults
- What is adult-onset Still disease
- Pathogenesis of adult-onset Still disease
- Fever and rash pattern in adult-onset Still disease
- Salmon-pink rash and its microscopic appearance
- Arthritis and organ involvement in adult-onset Still disease
- Differential diagnosis of adult-onset Still disease
- Treatment of adult-onset Still disease
Relapsing Polychondritis
An inflammatory disease of cartilage
- What is relapsing polychondritis
- Diagnostic pathway for relapsing polychondritis
- Epidemiology and cause of relapsing polychondritis
- Ear involvement in relapsing polychondritis
- Erythema and swelling of the ear, sparing the earlobe
- Nose, airway and joint involvement
- Skin findings and overlap syndromes in relapsing polychondritis
- Differential diagnosis of nasal deformity or destruction
- Differential diagnosis of nasal deformity or destruction
- Differential diagnosis of nasal deformity or destruction (continued)
- Treatment of relapsing polychondritis
Sjogren Syndrome
Autoimmune attack on the glands that make tears and saliva
- What is Sjogren syndrome
- 2016 ACR/EULAR classification criteria for Sjogren syndrome
- ACR/EULAR 2016 classification criteria - who the criteria apply to
- ACR/EULAR 2016 classification criteria - scored items
- Epidemiology of Sjogren syndrome
- Pathogenesis of Sjogren syndrome
- Dry eyes and dry mouth: how they present
- Downstream problems from chronic dryness
- Skin findings in Sjogren syndrome
- Palpable and non-palpable purpura in Sjogren syndrome
- Hypergammaglobulinemic purpura in Sjogren syndrome
- Vasculitis and other organ involvement
- Laboratory findings and diagnosis in Sjogren syndrome
- Treatment of Sjogren syndrome
- Xerostomia – patient instructions
- Xerostomia – patient instructions (continued)
- Xerostomia – patient instructions (continued)
- Xerostomia – patient instructions (continued)
- Xerostomia – patient instructions (continued)
- Xerostomia – patient instructions (continued)
- Xerostomia – patient instructions (continued)
- Xerostomia – patient instructions (continued)
Mixed Connective Tissue Disease
Overlapping features of several autoimmune diseases
- What is mixed connective tissue disease
- Epidemiology and pathogenesis of MCTD
- Skin and internal organ findings in MCTD
- Diagnosis and treatment of MCTD
Extra-Articular Manifestations of Rheumatoid Arthritis
Skin signs of rheumatoid arthritis, beyond the joints
- What is rheumatoid arthritis, briefly
- Pathogenesis of rheumatoid arthritis
- The main extra-articular signs of RA
- Rheumatoid nodules
- Periarticular rheumatoid nodules
- Rheumatoid nodules in a second patient with rheumatoid arthritis
- Accelerated rheumatoid nodulosis
- Methotrexate-induced nodulosis
- Rheumatoid nodule under the microscope
- Rheumatoid vasculitis
- Working up suspected rheumatoid (medium-vessel) vasculitis
- Bywaters lesions and Felty syndrome
- Neutrophilic skin diseases in rheumatoid arthritis
- Pyoderma gangrenosum in a patient with rheumatoid arthritis
- Differential diagnosis of rheumatoid nodules and vasculitis
- Working up a palisaded necrotizing granuloma
- Treatment of rheumatoid arthritis skin disease
Autoinflammatory Diseases
Inherited diseases of an overactive innate immune system
- What are autoinflammatory diseases
- The skin patterns seen in autoinflammatory disease
- The role of interleukin-1 in autoinflammatory disease
- How loss of the IL-1 and IL-36 brakes causes disease
- How the DIRA and DITRA gene defects unleash inflammation
- IL-1 blocking drugs used in autoinflammatory disease
- Where anakinra, rilonacept and canakinumab act on IL-1 signaling
- Range of cutaneous findings in inherited autoinflammatory diseases
- Range of cutaneous findings in inherited autoinflammatory diseases
- Range of cutaneous findings in inherited autoinflammatory diseases (continued)
- Range of cutaneous findings in inherited autoinflammatory diseases (continued)
- Range of cutaneous findings in inherited autoinflammatory diseases (continued) (continued)
- Range of cutaneous findings in inherited autoinflammatory diseases (continued)
- Range of cutaneous findings in inherited autoinflammatory diseases (continued) (continued)
- Range of cutaneous findings in inherited autoinflammatory diseases (continued)
- Range of cutaneous findings in inherited autoinflammatory diseases (continued) (continued)
- Range of cutaneous findings in inherited autoinflammatory diseases (continued)
- Range of cutaneous findings in inherited autoinflammatory diseases (continued) (continued)
- Genetics of the inherited autoinflammatory diseases
- Inflammasomopathies and IL-1-family conditions (genetics and treatment)
- Aberrant NF-kB/TNF activity and type I interferonopathies
- SAVI, ubiquitination disorders, and other mechanisms
- The look of inherited autoinflammatory disease on skin
- Cutaneous manifestations of inherited autoinflammatory disorders
- SAVI and Aicardi-Goutieres syndrome
- Digit resorption and nail loss in STING-associated vasculopathy
- Pernio-like lesions in Aicardi-Goutieres syndrome
- Where autoinflammatory disease overlaps with common skin disease
- Key takeaways
- References
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- References (continued)
- Dermatology, 5th Edition (2-Volume Set)