Connective Tissue and Bullous Disorders Lecture Powerpoint

Question 1

Connective tissue diseases have ___ symptoms and affect ____ body systems

Answer 1

Varying, almost all

Question 2

Lupus erythematosus and what 7 things does it clinically present with?

Answer 2

Chronic multisystem autoimmune disease of skin, joints, and internal organs that presents with fatigue, myalgia, low grade fever, mucocutaneous symptoms, raynauds, weight changes, and a malar rash**

Question 3

Malar rash

Answer 3

Lesion that occurs when systemic lupus is active, a classic rash in sun exposed areas that spares nasolabial folds and knuckles and is nonpruritic

Question 4

Discoid rash

Answer 4

Lesion that indicates systemic lupus, appears like psoriasis with thickened scaly patches on ears, predisposition to future skin cancer, spares the knuckles

Question 5

Musculoskeletal signs of lupus

Answer 5

Symmetrical pain and swelling

Question 6

Neurological signs of lupus

Answer 6

Migraine headaches that do not respond to treatment, seizures

Question 7

Sicca syndrome/sjogrens

Answer 7

Associated autoimmune disease occurs with lupus, indicated by dry eyes, sandy feeling in eye, dry mouth

Question 8

Drug induced lupus syndrome

Answer 8

Autoimmune reacion associated with chronic use of specific medications, typically see improvement upon withdrawal of offending drug

Question 9

Labs for lupus (3)

Answer 9

• CBC
• ESR/CRP
• ANA

Question 10

Positive ANA

Answer 10

A cardinal feature of lupus erythmatosus, indicates 90% likely positive, chances of false positive increases in older patients

Question 11

Dermatomyositis

Answer 11

Rare inflammatory muscle and skin disease that typically presents with a violet colored red rash that does not spare the knuckles followed by proximal skeletal muscle weakness and inflammation, gottron’s papules are pathognomonic finding, often autoimmune and co-occurs with malignant tumor (and resolves with their clearance)

Question 12

Gottron’s papules

Answer 12

Round 1cm red flat topped papules that occur ofer the knuckles and alongside the sides of fingers pathognomonic for dermatomyositis

Question 13

Labs for dermatomyositis (3)

Answer 13

• CK levels
• Muscle biopsy
• ANA

Question 14

Scleroderma

Answer 14

Unknown etiology rare chronic disease that causes abnormal tissue fibrosis and hardening of either skin and subQ (crest syndrome) or systemic organs

Question 15

Signs of Crest syndrome (5)

Answer 15

• telangiectasia
• raynauds
• esophageal dysfunction
• sclerodactyly (claw hand)
• Ca2+ deposit buildup

Question 16

Scleroderma diagnosis and treatment

Answer 16

• clinical

- none, supportive therapy

Question 17

Pemphigus vulgaris

Answer 17

Group of rare blistering diseases of skin and mucus membranes, fatal unless treated with immunosuppressive drugs and corticosteroids due to autoimmune origin, occurs almost exclusively in middle aged older people

Question 18

Bullous Pemphigoid

Answer 18

Rare skin condition that is common in older adults that causes fluid filled bliters on area of skin but not any on oropharyngeal leasions, low mortality and often self limiting, treated by oral prednisone

Question 19

Dermatitis herpetiformis

Answer 19

Pruritic (distinguishing feature) group of erythematous papulovesicles often seen with gluten sensitvity and associated with thyroid disorders, treated with dapsone and gluten free diet