Font size
WorksheetsChapter 62: Dermatomyositis
Total questions: 41
Worksheet time: 41mins
All of the following are true about clinically amyopathic dermatomyositis, EXCEPT:
Approximately 20% of dermatomyositis patients
DM rash for 6 months without weakness by history or PE
Normal strength but abnormal muscle enzymes
Normal strength and normal muscle enzymes
None of the above
Which of the following are considered as major criteria for cutaneous dermatomyositis (select all possible answers):
Heliotrope rash
Gottron sign
Shawl sign
Gottron papules
Holster sign
All of the following are true about scalp involvement in dermatomyositis, EXCEPT:
Pruritus is often mild or non-existent
One of the most commonly involved sites
Presents as a linear band just above and below the frontal hairline
Not specific for dermatomyositis
Identify the sign.
This pink to violet eruption can involve other areas, including which of the following (select all possible answers):
Cheeks
Neck
Scalp
Forehead
Nose
Identify the sign.
(a)
Identify the sign: confluent violaceous erythema on the sun-exposed areas of the lower anterior neck and anterior chest.
(a)
Identify the sign: violaceous to pink papules over the IP and MCP joints.
(a)
Identify the sign.
This symmetric macular violaceous erythema can be seen over the following areas (select all possible areas):
MCP joints
IP joints
Patellae
Olecranon process
Lateral malleoli
Identify the sign: violaceous erythema and poikiloderma on the lateral hips and lateral thighs.
(a)
Identify the sign: hyperkeratosis and fissuring along the medial thumb and lateral second and third digits.
QUESTION:
Dermatomyositis patients with this sign show increased prevalence of what systemic disease (spell out).
(a)
Which vasculopathic manifestation is highly suggestive of dematomyositis over other connective tissue disorders?
Gingival telangiectasia
Livedo reticularis
Ulceration
Proximal nailfold erythema
All of the following are true regarding ulceration in dermatomyositis, EXCEPT:
Associated with positive anti-MDA5 antibodies
Necrotic ulcerations raises the concern for malignancy
Worsening ulceration may be a sign of worsening ILD
Often affects the flexor surfaces of the extremities
This sign is associated with positive anti-transcriptional intermediary factor 1γ (TIF1-γ) antibodies.
Identify the sign.
(a)
Cutaneous disease activity in dermatomyositis is characterized by which of the following signs (select all possible answers):
Erythema
Itch
Induration
Scale
Ulceration
Identify this sign: reticulated white macules surrounded by telangiectatic red macules.
All of the following are true about this skin finding in dermatomyositis, EXCEPT:
It is a sign of substantial inflammation
It is also seen in other connective tissue diseases like lupus
It is frequently seen over the bitemporal hairline
It doesn't necessarily follow patterns of sun exposure
Poikiloderma is a sign of significant damage after long-standing disease activity. It is characterized by which of the following (select all possible answers):
Atrophy
Hypopigmentation
Hyperpigmentation
Telangiectasias
All of the following are true about calcinosis in dermatomyositis, EXCEPT:
It is more prevalent in juvenile DM
It most frequently presents as digital calcinosis
Positive anti-nuclear matrix protein 2 (NXP-2) antibodies are associated with increased risk
Vascular insufficiency or damage is involved in its pathogenesis
All of the following are risk factors for development of calcinosis in juvenile DM, EXCEPT:
Longer disease duration
Younger age of onset
Sustained disease activity
Absence of internal organ involvement
Anti-MDA5 antibodies are associated with all the following clinical findings in dermatomyositis, EXCEPT:
Ulceration
Violaceous patch over the hard palate
Reticular pattern of erythema on the palms
Calcinosis
Severe alopecia
All of the following are true about interstitial lung disease in dermatomyositis, EXCEPT:
Leading cause of morbidity and mortality
Most common pulmonary manifestation
DM with anti-MDA5 antibodies have increased risk of developing ILD
Most common radiographic pattern is cryptogenic organizing pneumonia
Which diagnostic tool is necessary for establishing the diagnosis of interstitial lung disease in dermatomyositis?
6-minute walk test
Pulmonary function tests
Chest x-ray
High-resolution CT scan of the chest
Which radiographic pattern of interstitial lung disease is associated with poor prognosis?
Basilar and peripheral ground-glass opacities
Subpleural sparing
Cryptogenic organizing pneumonia
Diffuse alveolar damage
What is the gold standard for diagnosis of pulmonary arterial hypertension in dermatomyositis?
Pulmonary function tests
Transthoracic echocardiography
High-resolution CT scan
Right heart catheterization
All of the following are true about muscle involvement in dermatomyositis, EXCEPT:
Typical presentation is symmetrical proximal weakness
About 80% of patients develop weakness within the first year of symptom onset
Myalgia is always accompanied by weakness and is seen in 50% of patients
Distal muscle weakness is associated with anti-NXP2 antibodies
All of the following are true about gastrointestinal involvement in dermatomyositis, EXCEPT:
Uncommon manifestation of adult DM
Associated with more severe disease
Life threatening sequelae include ulceration and perforation
Results from vasculopathy affecting the bowel wall
Subclinical cardiac muscle involvement can be detected using this useful biomarker:
(a)
Which of the following factors is associated with an INCREASED RISK for malignancy in dermatomyositis?
Increasing age
Male gender
Cutaneous necrosis
ILD
Arthritis
Which of the following factors is considered PROTECTIVE for malignancy in dermatomyositis?
Dysphagia
Rapid onset of myositis
Raynaud phenomenon
Arthritis
Which of the following genes are associated with the development of dermatomyositis (select all the possible answers):
HLA-B8
BLK gene
TYK2 gene
HLA-B27
All of the following are true about the pathogenesis of dermatomyositis, EXCEPT:
Immune-mediated disorder of both innate and adaptive immunity
Interface dermatitis with keratinocyte injury in the skin
Atrophy, degeneration, and regeneration of muscle fibers
Decreased levels of interferon (IFN)-induced genes and proteins
Weakness symptoms of DM can be attributed to the following pathogenic factors, EXCEPT:
Endoplasmic reticulum stress response
Activation of NF-kB signaling pathways
Decreased reactive oxygen species
Mitochondrial dysfunction
What physical signs are sensitive for dermatomyositis (select all possible answers):
Lateral digit hyperkeratosis
Scalp erythema and dyesthesia
Microscopic periungual telangiectasia
Ovoid palatal patch
Gottron papules
What physical signs are specific for dermatomyositis (select all possible answers):
"Red-on-white" patches
Ovoid palatal patch
Grossly visible periungal telangiectasias
Gottron papules
Microscopic periungal telangiectasias
Which serum muscle enzymes can be used to evaluate for myositis early on in the course of dermatomyositis (select all possible answers):
Creatine kinase
AST
ALT
Aldolase
Lactate dehydrogenase
Serum muscle enzymes can be falsely elevated in the following situations (select all possible answers):
10-14 days after strenuous activity
Liver disease
Hemolysis of blood sample
Kidney disease
What alternative biomarker can be used for DM patients with anti-MDA5 antibodies that reflects the clinical response of ILD?
(a)
All of the following are true about the histopathologic findings of dermatomyositis in the skin, EXCEPT:
Interface dermatitis
Dense infiltrates
Increased dermal mucin
Epidermal atrophy
Basement membrane thickening
All of the following are true about histopathology of the muscle (muscle biopsy) in dermatomyositis, EXCEPT:
Required to establish the diagnosis of DM
Highest yield if performed within 2 weeks of beginning any immunomodulatory therapy
Histopathology shows perifascular atrophy and degenerating and regenerating myofibers
False-negative results can be due to patchy inflammation
Which of the following are considered the major causes of death in dermatomyositis (select all possible answers):
Malignancy
Pulmonary disease
Cardiac disease
Infection
Which autoantibody is considered a risk factor for mortality?
anti-MDA5
anti-TIFI-y
anti-NXP-2
antisynthetase
All of the following are true about cancer screening in dermatomyositis, EXCEPT:
30% of DM patients have an associated malignancy
Routine age-appropriate screening is recommended on initial diagnosis
More aggressive screening may detect occult malignancies within the higher risk period (2-3 years from diagnosis)
Blind rescreening should be considered with difficult to control or flare of disease
