2/14 Immunologic Skin Disorders Flashcards
What is this disease called?
lupus erythematosus - acute form
What is this called?
lupus erythematosus - subacute form
What is this disease called?
lupus erythematosus - chronic form
What disease are these findings associated with?
Bonus: What is each one called?
Dermatomyositis
What disease is this associated with?
Scleroderma - morphea
What disease are these symptoms associated with?
Scleroderma - CREST
- Calcinosis cutis
- Raynaud’s phenomenon
- Esophageal dysmotility - sclerosis of LES due to chronic reflux
- Sclerodactaly
- Telangiectasias
What disease are these findings associated with?
Scleroderma - Systemic sclerosis
What is lupus erythematosus?
What forms are there?
Multi-system disease that often has prominent skin involvement
autoantibodies are directed against components of cell nucleus (antinuclear antibodies, ANA)
deposition of immune complexes in various tissues cause complement activation and tissue damage
3 forms: acute, subacute, and chronic
What is the clinical presentation of lupus erythematosus - acute form?
Which antibody is often found in these?
- butterfly rash (malar rash)
- most common rash present in SLE patients (90% of patients)
- flare of skin disease may reflect activity of disease in other organ systems
- anti-dsDNA antibodies
- often photosensitive
What is the clinical presentation of lupus erythematosus - sub-acute form?
What may cause this form?
What antibodies are often present? What is unique about one of these antibodies?
- marked photosensitivity
- anti-Ro and anti-La antibodies
- may be primarily a skin problem or in the setting of SLE (50% of patients)
- may be drug induced (HCTZ, NSAIDS, terbinafine, diltiazem)
- *neonatal lupus** is a risk for children born to mothers with SCLE sine anti-Ro abs can cross the placenta
- *-** transient skin rash on face, around eyes, trunk
- risk of complete heart block
- may require pacemaker
- *-** may have associated hepatobiliary disease, thrombocytopenia
What is the clinical presentation of lupus erythematosus - chronic form?
- discoid lupus
- can be purely cutaneous (CCLE) or in the setting of SLE (10% of patients); low risk of progression to SLE
- can cause significant scarring, especially because it favors the face scalp
How do you treat lupus erythematous?
- sun protection
- corticosteroids
- antimalarials (hydroxychloroquine)
- systemic immunosuppressants
- retinoids
- thalidomide
What is a risk of using antimalarials to treat lupus erythematous?
retinal toxicity
What are the 3 major forms of dermatomyositis?
- polymyositis: only muscle
- dermatomyositis: skin and muscle involvement
- amyopathic dermatomyositis: only skin
Dermatomyositis affects both children and adults - how are they different?
Children - 7.6 yrs
- malignancy is NOT typically associated
- frequently develop calcinosis and vasculitic skin lesions
Adults - 52 yrs; with female preponderance
- associated with malignancies (lung, breast, ovaries, GI tract) in 10-50% adults that may precede onset of dermatomyositis symptoms
- may be associated with:
o cutaneous vasculitis
o “malignant erythema”
o ulcerations
o skin disease refractory to treatment
- vigilant screening mandated
What are some of the lab findings in Dermatomyositis?
- symmetrical proximal muscle weakness
- abnormal muscle biopsy
- increased CPK (skeletal muscle enzymes)
- abnormal electromyogram (EMG)
- characteristic skin lesions
- ANA may be negative; may have antisynthetase ab (inc. Jo-1)
- associated with pulmonary disease
How is dermatomyositis treated?
skin disease - topical corticosteroids, antimalarials
muscle disease - systemic corticosteroids; steroid-sparing agents (methotrexate, azathioprine) may be added
What is Scleroderma?
What is involved?
How does it evolve?
sclerosis (thickening) of the skin and other tissues/multiple other organ systems, especially the lungs and GI tract
pathogenesis is** incompletely understood**
pathogenic steps thought to include:
- *- vascular damage
- activation of T cells
- altered connective tissue production by fibroblasts**
What 3 disease is scleroderma associated with?
Morphea
CREST
Systemic sclerosis
What is morphea?
What is the progression of disease?
How is it treated?
- Scleroderma localized to the skin and subcutaneous tissues (may initially be in lilac region, but may involve joints and bone)
- hyperpigmented, but over time, becomes sclerotic and may become ivory white
- disease usually progresses for several years, then regresses (“burns out”)
Treatment options often ineffective, but can include:
- corticosteroids
- Phototherapy (PUVA)
- vitamin derivatives (Vits A and D)
- immunosuppressive agents
What is CREST?
What antibody is usually present?
- subset of patients with limited scleroderma
- Calcinosis cutis
- Raynaud’s phenomenon
- Esophageal dysmotility – narrowing/sclerosis of LES due to chronic reflux
- Sclerodactaly
- Telangiectasias
- anticentromere antibodies often present
What is Systemic sclerosis?
What is the progression of disease? What are some of the findings associated with it?
either limited or diffuse scleroderma in addition to involvement of multiple internal organs; most severe form
cutaneous findings:
- diffuse hyperpigmentation
- telangiectasias
- sclerodactaly
- digital ulcers
- Raynaud’s phenomenon
- *internal findings (REALS)**
- renal disease
- arthralgia
- esophageal and bowel involvement
- lung disease
- sicca symptoms
diffuse cutaneous involvement generally worse prognosis (often fatal; 10 year survival = 20%)
What is Systemic sclerosis?
How is it diagnosed and treated?
- diagnosis based on clinical presentation, but skin biopsy can confirm cutaneous component
- ANA usually positive (antinucleolar antibody pattern most specific)
- antibodies to Scl 70 (topoisomerase I): often associated w. pulmonary fibrosis
Skin disease often poorly responsive to treatment
- immunomodulators (methotrexate, cyclophosphamide, steroids, thalidomide, UV light, etc.)
- Ca channel blockers for Raynaud’s
- IV alprostadil (PGE1) for refractory Raynaud’s
- Prostacyclin for pulmonary HTN
BAD = ACE inhibitors has made lung disease rather than renal crisis main cause of death
What are the diagnostic features of SLE?
MD SOAP BRAIN
- *Malar rash** – butterfly rash, sparing of nasolabial folds
- *Discoid rash** – basement membrane involved, may cause scarring
- *Serositis** – pleuritis/pericarditis
- *Oral ulcers**
- *Antinuclear antibody (ANA)** – very sensitive test
- *Photosensitivity** – skin rash to sunlight
- *Blood** – haemolytic anaemia, leukopaenia, thrombocytopaenia
- *Renal** disorder – proteinuria and cell casts
- *Arthritis** – symmetrical, involving 2+ small or large peripheral joints
- *Immunological** disorder – anti-dsDNA
- *Neurological** – seizures, psychosis
ALSO Liebmann Sacks
