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 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 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%)
