dermatomyositis

Question 1

age of onset for dermatomyositis?

Answer 1

bimodal (5-14 yo and 45-65)

Question 2

Pathogenesis of dermatomyositis?

Answer 2

• environmental factors (malignancy, viral infections) trigger an immunde-mediated process in genetically susceptible individuals (various HLA’s and TNF alpha 308A polymorphism in juvenile onset)

Question 3

What are the genetic susceptibilities of Dermatomyositis?

Answer 3

various HLA’s and TNF alpha 308A polymorphism in juvenile onset

Question 4

Drugs implicated in drug induced Dermatomyositis:

Answer 4

• hydroxyurea (most common, >50%)
• Statins
• D-penacillamine, cyclophosphamide, BCG vaccine, TNF-alpha inhibitors

Question 5

Describe the muscle findings in Dermatomyositis.

Answer 5

• slowly progressive symmetric, proximal muscle weakness typically affecting the shoulders, hip girdle, and neck flexors (difficulty walking upstairs, brushing hair, standing from seated position)

Question 6

Is muscle inovlement in Dermatomyositis painful?

Answer 6

painless (no mylagias)

Question 7

What muscular organs can be involved in Dermatomyositis?

Answer 7

• esopahageal muscles–> dysphagia
• Cardiac muscle–> EKG abnormalities, CHF, complete heart block
• diaphragm–> rare but life-threatening

Question 8

What are the classic skin findings of Dermatomyositis?

Answer 8

• gottron’s papules (pathognomonic): lichenoid papules overlying knuckles
• symmetric confluent macular violaceous erythema w/ malar involvement (vs lupus)
• heliotrope sign (due to inflammation of underlying orbicularis oculi muscle, not skin!) of eyelids +- periorbital edema
• holster sign (lateral thighs
• Macular erythema Overlying joints (Gottron’s sign)

- photodistributed poikiloderma

• V-sign or shawl sign of chest/upper back
• mechanics hands

Question 9

What are the hand findings in Dermatomyositis and what are they a/w?

Answer 9

mechanics hands (rough hyperkeratosis and fissuring of the lateral and palmar side of fingers)

• a/w anti-sythetase syndrome

Question 10

What nail changes can you see in Dermatomyositis?

Answer 10

• Ragged cuticles

- proximal nailfold w/ dilated capillary loops alternating with areas of vessel dropout

- periungual erythema

Question 11

What is a symptom of Dermatomyositis that can help differentiate from lupus?

Answer 11

severe pruritis (often on scalp) , lupus not often itchy

Question 12

what skin finding can you see in dermatomyositis (less common, but more frequently seen in juvenile DM)?

Answer 12

calcinosis cutis

Question 13

_____ is an uncommon skin finding of dermatomyositis that is a/w anti-CADM-140 antibodies.

Answer 13

Palmar papules (erythematous palmar papules overlying hyperkeraosis/ulcerartion; painful (unlike gottron’s papules)

Question 14

Vasculitis occurring in adult with dermatomyositis is a/w _________

Answer 14

Malignancy

Question 15

Juvenile dermatomyositis with severe systemic vasculitis is called ______

Answer 15

Banker variant JDM

Question 16

Banker variant JDM prognosis and presentation:

Answer 16

• this is juvenile Banker variant JDM with severe vasculitis
• severe course and poor response to tx
• presents with cutaneous ulcerations, muscle infarctions, GI involvement, widespread calcinosis

Question 17

What pulmonary disease is a/w Dermatomyositis?

Answer 17

interstitial lung disease

Question 18

rapidly progressive interstitial lung disease in Dermatomyositis is a/w what two antibodies?

Answer 18

• anti-sythetase

- anti-CADM-140

Question 19

Clinically amyopathic Dermatomyositis presents with _______ and is a/w _____

Answer 19

• classic skin findings but no clinical muscle disease
• a/w ILD and Anti-CADM-140 antibodies

Question 20

Cancer associated Dermatomyositis is a/w:

Answer 20

- increased age

• rapid disease onset

- skin necrosis

- anti-p155/140 antibodies

Question 21

Most common cancers a/w Dermatomyositis are:

Answer 21

• ovarian and GI (classic exam answer)
• nasopharyngeal carcinoma in asians
• breast, lung, pancreatic, non-hodgkins

Question 22

Risk for most cancers a/w Dermatomyositis returns to normal after _____ (how long?)

Answer 22

5 years

Question 23

Anti-synthetase syndrome presents with:

Answer 23

• acute onset disease
• constitutional symptoms
• raynauds phenomenon
• mechanics hands
• arthritis
• interstitial lung disease

- anti-synthetase antibodies

Question 24

Is juvenile Dermatomyositis a/w malignancy risk?

Answer 24

NO!

Question 25

_____ antibodies are a/w calcinosis cutis and contractures in juvenile Dermatomyositis.

Answer 25

• Anti-p140

Question 26

_____ antibodies are a/w interstitial lung disease in kids with Juvenile Dermatomyositis.

Answer 26

Anti-CADM-140

Question 27

What are the two variants of juvenile Dermatomyositis?

Answer 27

Classic JDM (Brunsting variant)

• gradual onset of classic skin and muscle disease, calcinosis cutis, corticosteroid responsive

Vasculopathic/ulcerative JDM (Banker variant)

• rapid onset of severe muscle disease, vasculitis w/ systemic symptoms, and poor prognosis

Question 28

Histopath of Dermatomyositis:

Answer 28

• subtle vacuolar interface
• rare scattered necrotic keratinocytes
• epidermal atrophy
• sparse PV/PA lymphocytic inflam

- massive dermal mucin

Question 29

Lab testing to order for patient with Dermatomyositis:

Answer 29

• CK/aldolase (increased muscle enzymes)
• EMG
• muscle biopsy= gold standard for dx
• antibodies

Question 30

First line therapy for dermatomyositis with skin-limited disease?

Answer 30

photoprotection,

topical CS,

TCI,

+- antimalarials

Question 31

What screening visits/labs should be done for f/u after diagnosing Dermatomyositis?

Answer 31

• re-check muscle enzymes and muscle strength q 2-3 months (if muscle involvement, start systemic steroids)
• physical exam q4-6 months for first 2-3 years to screen for malignancy

Question 32

first line tx for skin+muscle involvment in Dermatomyositis?

Answer 32

• systemic corticosteroids, MTX, azathioprine

Question 33

most common cause of death in Dermatomyositis in adults? Kids?

Answer 33

• malignancy in adults (also ischemic heart disease and pulmonary disease)
• kids usually have favorable outcomes with minimal sequelae if treated early and adequate therapy with CS.

Question 34

Drug induced Dermatomyositis is split into_____

Answer 34

• hydroxyurea induced and non-hydroxyurea induced

Question 35

Hydroxyurea induced dermatomyositis has a _____ (short/long) latency period and myositis is (present/absent)

Answer 35

long latency (60 months)

- myositis is never seen!!!!

• this is opposed to non-hydroxyurea induced Dermatomyositis which occurs within 2 months and 80% have myositis

Question 36

drug induced Dermatomyositis resolves after _____

Answer 36

• 1-2 months after discontinuation of med