Connective Tissue & Bullous Disorders of the Skin - MD Flashcards
What are characteristics of Lupus Erythematosus?
- Chronic, multisystem Dz
- Autoimmune
- Genetic predisposition
- Women > men
- MC in Af. Americans & Hispanics
- Onset 16-55 y.o.
- Triggers include UV light, EBV, smoking
What are S/S of SLE?
- Fatigue (MC)
- Myalgia/weakness
- Low grade fever
- Photosensitivity, alopecia, oral/nasal ulcerations
- Raynauds
- Weight loss/gain
What are the 3 different types of SLE mucocutaneous rashes?
1) Malar rash: occurs when SLE is active, characterized by edema/photosensitivity
2) Discoid Rash (looks like psoriasis): erythematous & chronic
3) Subacute Cutaneous: Erythematous papules/plaques with slight scaling mimicking psoriasis
What are characteristics of acute cutaneous LE?
- occurs on sun-exposed areas
- Associated with systemic Dz
- Nonpuritic, erythematous rash on exposed parts of the body
- Classic “butterfly” or “malar” rash on face
What are characteristics of DLE (Discoid Lupus)?
-like acute cutaneous LE but lower incidence of systemic disease. and can lead to severe scarring
What are the characteristics of subacute cutaneous LE?
- Falls b/w chronic destructive DLE & erythema of acute cutaneous LE
- Two forms → papulosquamous & annular
- lesions heal without scarring
- associated with arthralgia/myalgia
What positive lab result is the cardinal feature in SLE diagnosis since it occurs in over 90% of cases?
Positive for the presence of ANA
What are aspects of a treatment plan for a patient with LE?
- modifying CV risk factors (leading cause of morbidity and mortality)
- Avoid sunlight
- Calcium + Vit D
- Hydroxychloroquine (Plaquenil) → protects against organ damage
- Methotrexate for persistent arthritis
- Immunosuppresive Tx
What is the difference between polymyositis and dermatomyositis?
Dermatomyositis is inflammatory muscle and skin Dz
Polymyositis is same but without skin involvement
What are characteristics of Dermatomyositis?
1) Heliotrope erythema of the eyelids
- pathognomonic
- Periorbital edema and violet discoloration
2) Gottron’s papules:
- pathognomonic
- smooth, violaceous-to-red, flat topped papules, occuring over the knuckles, along with sides of fingers.
What findings would assist in making a Dermatomyositis Dx?
- Symmetrical weakness of limb girdle muscles
- Elevated muscle enzyme levels
- Myopathic pattern on EMG
- Muscle Biopsy evidence on inflammation
- Skin rash
What is the Tx for dermatomyositis?
- Steroid taper (first line)
- Azathioprine/ Methotrexate (second line)
- Exercise
What are characteristics of limited scleroderma (CREST Syndrome)?
C – Calcinosis R – Raynaud's phenomenon E – Esophageal dysfunction S – Sclerodactyly T – Telangiectasia
What are the most common manifestations of Systemic Scleroderma?
Most common symptoms:
- Raynaud’s,
- diffuse swollen hands,
- arthralgias/myalgias,
- fatigue
What is the Tx plan for a patient with scleroderma?
- Treat symptoms
- No disease modifying agents available