Connective Tissue Diseases Flashcards
What population is more likely to have independent cutaneous lupus erythematosus (CLE)?
What antibodies are associated with CLE?
What is associated with increased activity and resistance?
- Women
- Peak incidence in 4th decade
- Skin types 4-6 (and more severe)
Anti-Ro, Anti-dsDNA, Anti-Sm
Smoking
What forms of lupus are more photosensitive?
active and subacute cutaneous lupus (ACLE, SCLE)
Name this dermatological diagnosis
Discoid lupus
Name this dermatological diagnosis
Discoid lupus
What is the % of discoid lupus that is localized?
What is the % of discoid lupus that is generalized?
5-10%
15-28%
Name that dermatological diagnosis
What percentage is drug-induced?
What are common drug-induced triggers?
Subacute cutaneous lupus erythematosus (SCLE)
25% (almost all drug induced is SCLE)
- PPIs
- Antihypertensives
- Terbinafine
- Statins
- Diuretics
Name this dermatological diagnosis
Acute cutaneous lupus erythematosus (ACLE)
What findings are seen on DIF for cutaneous lupus erythematosus?
- “lupus band test”, non-specific so not recommended
- Linear or granular IgG, IgM, IgA and complement at the dermoepidermal junction
What features heighten suspicion for cutaneous lupus erythematosus?
- Mucocutaneous and inflammatory symptoms
- Proteinuria
- Anti-ds DNA and Anti-Sm antibodies
- Low complement levels
What are the general treatment recommendations for cutaneous lupus erythematosus?
Localized/limited disease?
Disseminated/active disease?
General Management
- Lifestyle changes - photoprotection, smoking cessation
- Vitamin D supplementation
- Consider possibility of drug-induced CLE or SLE
Localized/Limited Disease
- Potent topical corticosteroids (dermovate x 1/12)
- TCIs (protopic 0.1%)
- Consider intralesional triamcinolone
Disseminated/active Disease
- FIRST LINE
- Hydroxychloriquine (HCQ)
- +/- Mepacrine
- monotherapy if resistant to standard dosing (double) or combo therapies/others are contraindicated
- Chloroquine when HCQ or mepacrine ar not tolerated or ineffective
- Short-term tapering courses of systemic corticosteroids for flares
- SECOND LINE
- MTX
- MMF
- Dapsone
- Acitretin for CLE and hyperkeratotic DLE resistant to topicals and antimalarials
- THIRD LINE
- Clofazimine
- Biologics (belimumab, rituximab)
- IVIG
- Thalidomide
- Lenalidomide
What percentage of individuals have muscle weakness at time of presentation with polymyositis? with dermatomyositis?
What are the clinical criteria for diagnosis?
90%; 50%
- Dermatomyositis: characteristic skin rash (heliotrope with Gottron’s)
- Muscle biopsy abnormalities typical of myositis
- Typical EMG features of myositis
- Elevation of skeletal muscle enzymes
- Symmetrical proximal muscle weakness
What investigations would you order for suspected dermatomyositis?
When is the highest risk for malignancy with DM? Types?
What is MDA5 associated with?
What is TIF1 associated with?
- CK
- Extended myositis panel
- CXR (PFTs and CT if suspicion of fibrosis)
- Muscle biopsy
- Skin biopsy
- Malignancy screen (especially if high risk antibody)
In the first 5 years; GI/GU solid tumours
Skin disease and Lung disease
Skin disease, calcinosis and malignancy
What is the treatment for dermatomyositis?
- Photoprotection
- Potent topical steroids
- Antimalarials (HCQ +/- mepacrine)
- Steroid-sparing agents - MTX vs AZA
- Other agents
- IVIG
- MMF
- Rituximab
- WHEN MUSCLE DISEASE PRESENT: Systemic steroids slowly tapered over 12 months
Name this dermatological diagnosis
En coup de sabre - localized scleroderma (morphea)
Name this dermatologic diagnosis
Localized scleroderma (morphea)
What 2 types of morphea do these pictures depict?
How do you differentiate?
Generalized and Circumscribed
Generalized = ≥3 sites
Circumscribed = ≤2 sites
Name this dermatological diagnosis
Parry Romberg Syndrome
