Connective Tissue and Bullous Disorders Lecture Powerpoint Flashcards
Mike take me home
Connective tissue diseases have ___ symptoms and affect ____ body systems
Varying, almost all
Lupus erythematosus and what 7 things does it clinically present with?
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**
Malar rash
Lesion that occurs when systemic lupus is active, a classic rash in sun exposed areas that spares nasolabial folds and knuckles and is nonpruritic
Discoid rash
Lesion that indicates systemic lupus, appears like psoriasis with thickened scaly patches on ears, predisposition to future skin cancer, spares the knuckles
Musculoskeletal signs of lupus
Symmetrical pain and swelling
Neurological signs of lupus
Migraine headaches that do not respond to treatment, seizures
Sicca syndrome/sjogrens
Associated autoimmune disease occurs with lupus, indicated by dry eyes, sandy feeling in eye, dry mouth
Drug induced lupus syndrome
Autoimmune reacion associated with chronic use of specific medications, typically see improvement upon withdrawal of offending drug
Labs for lupus (3)
- CBC
- ESR/CRP
- ANA
Positive ANA
A cardinal feature of lupus erythmatosus, indicates 90% likely positive, chances of false positive increases in older patients
Dermatomyositis
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)
Gottron’s papules
Round 1cm red flat topped papules that occur ofer the knuckles and alongside the sides of fingers pathognomonic for dermatomyositis
Labs for dermatomyositis (3)
- CK levels
- Muscle biopsy
- ANA
Scleroderma
Unknown etiology rare chronic disease that causes abnormal tissue fibrosis and hardening of either skin and subQ (crest syndrome) or systemic organs
Signs of Crest syndrome (5)
- telangiectasia
- raynauds
- esophageal dysfunction
- sclerodactyly (claw hand)
- Ca2+ deposit buildup
Scleroderma diagnosis and treatment
- clinical
- none, supportive therapy
Pemphigus vulgaris
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
Bullous Pemphigoid
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
Dermatitis herpetiformis
Pruritic (distinguishing feature) group of erythematous papulovesicles often seen with gluten sensitvity and associated with thyroid disorders, treated with dapsone and gluten free diet