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Chapter 62 - Dermatomyositis Flashcards

1
Q

TABLE 62-1
Sontheimer’s Proposed Diagnostic Criteria for Cutaneous Dermatomyositis

Diagnosis of cutaneous dermatomyositis requires:
1. Presence of ___ major criteria or ___ major criterion and ___ minor criteria and
2. _____ consistent with cutaneous dermatomyositis

A

Diagnosis of cutaneous dermatomyositis requires:
1. Presence of two major criteria or one major criterion and two minor criteria and
2. Skin biopsy changes consistent with cutaneous dermatomyositis

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2
Q

TABLE 62-1
Sontheimer’s Proposed Diagnostic Criteria for Cutaneous Dermatomyositis

Major Criteria vs Minor Criteria

Heliotrope sign

A

Major Criteria

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3
Q

TABLE 62-1
Sontheimer’s Proposed Diagnostic Criteria for Cutaneous Dermatomyositis

Major Criteria vs Minor Criteria

Gottron papules

A

Major Criteria

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4
Q

TABLE 62-1
Sontheimer’s Proposed Diagnostic Criteria for Cutaneous Dermatomyositis

Major Criteria vs Minor Criteria

Gottron sign

A

Major Criteria

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5
Q

TABLE 62-1
Sontheimer’s Proposed Diagnostic Criteria for Cutaneous Dermatomyositis

Major Criteria vs Minor Criteria

Macular violaceous erythema involving various areas

A

Minor Criteria

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6
Q

TABLE 62-1
Sontheimer’s Proposed Diagnostic Criteria for Cutaneous Dermatomyositis

Major Criteria vs Minor Criteria

Nailfold capillary telangiectasia, hemorrhage-infarct

A

Minor Criteria

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7
Q

TABLE 62-1
Sontheimer’s Proposed Diagnostic Criteria for Cutaneous Dermatomyositis

Major Criteria vs Minor Criteria

Poikiloderma

A

Minor Criteria

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8
Q

TABLE 62-1
Sontheimer’s Proposed Diagnostic Criteria for Cutaneous Dermatomyositis

Major Criteria vs Minor Criteria

Mechanic’s hands

A

Minor Criteria

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9
Q

TABLE 62-1
Sontheimer’s Proposed Diagnostic Criteria for Cutaneous Dermatomyositis

Major Criteria vs Minor Criteria

Cutaneous calcinosis

A

Minor Criteria

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10
Q

TABLE 62-1
Sontheimer’s Proposed Diagnostic Criteria for Cutaneous Dermatomyositis

Major Criteria vs Minor Criteria

Cutaneous ulcers

A

Minor Criteria

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11
Q

TABLE 62-1
Sontheimer’s Proposed Diagnostic Criteria for Cutaneous Dermatomyositis

Major Criteria vs Minor Criteria

Pruritus

A

Minor Criteria

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12
Q

TABLE 62-1
Sontheimer’s Proposed Diagnostic Criteria for Cutaneous Dermatomyositis

Macular violaceous erythema
(each area counts as ___ minor criterion)

A

Macular violaceous erythema
(each area counts as one minor criterion)

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13
Q

TABLE 62-1
Sontheimer’s Proposed Diagnostic Criteria for Cutaneous Dermatomyositis

Macular violaceous erythema in the V-area of neck or upper chest

A

V-neck sign

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14
Q

TABLE 62-1
Sontheimer’s Proposed Diagnostic Criteria for Cutaneous Dermatomyositis

Macular violaceous erythema in the posterior neck or posterior shoulders

A

Shawl sign

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15
Q

TABLE 62-1
Sontheimer’s Proposed Diagnostic Criteria for Cutaneous Dermatomyositis

Macular violaceous erythema in the lateral thighs or hips

A

Holster sign

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16
Q

TABLE 62-2
Clinical Associations with Autoantibodies Associated with Dermatomyositis

Most frequent, up to 20%, anti-tRNA synthetase

A

Jo-1: histidyl

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17
Q

TABLE 62-2
Clinical Associations with Autoantibodies Associated with Dermatomyositis

AUTOANTIBODY

CLINICAL PHENOTYPE IN ADULT DERMATOMYOSITIS
Increased risk of ILD
PL-7: mild skin or muscle
disease
PL-12, KS, OJ: isolated ILD
Variable spectrum of findings of the antisynthetase syndrome: ILD, fever, arthritis, myositis, mechanic’s hands, Raynaud phenomenon

A

Anti-tRNA synthetase

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18
Q

TABLE 62-2
Clinical Associations with Autoantibodies Associated with Dermatomyositis

AUTOANTIBODY

CLINICAL PHENOTYPE IN ADULT DERMATOMYOSITIS
Hallmark cutaneous disease, good prognosis and response to therapy; relapses common; high CK values (>5000 U/L); rare ILD, cancer; not amyopathic

A

Anti–Mi-2

19
Q

TABLE 62-2
Clinical Associations with Autoantibodies Associated with Dermatomyositis

AUTOANTIBODY

CLINICAL PHENOTYPE IN ADULT DERMATOMYOSITIS
Increased cancer risk in adults
Severe cutaneous disease
Red-on-white skin lesions
Ovoid palatal patch
Low ILD risk
Low CK values (200–2000 U/L)

A

Anti–transcriptional intermediary factor (TIF1)-γ

20
Q

TABLE 62-2
Clinical Associations with Autoantibodies Associated with Dermatomyositis

AUTOANTIBODY

CLINICAL PHENOTYPE IN ADULT DERMATOMYOSITIS
High ILD risk, RP-ILD, especially in Asians
Skin ulceration, red palmar papules, alopecia, gingival pain
Arthritis
Often isolated high aldolase

A

Anti–melanoma differentiation–associated gene 5 (MDA5)

21
Q

TABLE 62-2
Clinical Associations with Autoantibodies Associated with Dermatomyositis

AUTOANTIBODY

CLINICAL PHENOTYPE IN ADULT DERMATOMYOSITIS
Increased cancer risk in adults
Increased risk of calcinosis
Peripheral edema, myalgia, severe dysphagia
Distal weakness

A

Anti–nuclear matrix protein 2 (NXP2)

22
Q

TABLE 62-2
Clinical Associations with Autoantibodies Associated with Dermatomyositis

AUTOANTIBODY

CLINICAL PHENOTYPE IN ADULT DERMATOMYOSITIS
Skin disease onset before myositis
May have severe skin disease
Dysphagia

A

Anti–small ubiquitin-like modifier activating enzyme (SAE)

23
Q

TABLE 62-2
Clinical Associations with Autoantibodies Associated with Dermatomyositis

AUTOANTIBODY

PHENOTYPE IN JUVENILE DERMATOMYOSITIS
Older onset
Antisynthetase syndrome with ILD, fever, nonerosive polyarthritis, Raynaud phenomenon, mechanic’s hands

A

Anti-tRNA synthetase

24
Q

TABLE 62-2
Clinical Associations with Autoantibodies Associated with Dermatomyositis

AUTOANTIBODY

PHENOTYPE IN JUVENILE DERMATOMYOSITIS
Classic skin disease
Myositis
Good therapeutic response; more likely to be in remission at 2 yr

A

Anti–Mi-2

25
Q

TABLE 62-2
Clinical Associations with Autoantibodies Associated with Dermatomyositis

AUTOANTIBODY

PHENOTYPE IN JUVENILE DERMATOMYOSITIS
Severe skin disease
Lipodystrophy

A

Anti–transcriptional intermediary factor (TIF1)-γ

26
Q

TABLE 62-2
Clinical Associations with Autoantibodies Associated with Dermatomyositis

AUTOANTIBODY

PHENOTYPE IN JUVENILE DERMATOMYOSITIS
ILD in Japanese cohort
Skin ulceration, arthritis, milder myositis

A

Anti–melanoma differentiation–associated gene 5 (MDA5)

27
Q

TABLE 62-2
Clinical Associations with Autoantibodies Associated with Dermatomyositis

AUTOANTIBODY

PHENOTYPE IN JUVENILE DERMATOMYOSITIS
Calcinosis, more severe myositis, poorer functional status

A

Anti–nuclear matrix protein 2 (NXP2)

28
Q

TABLE 62-4
Treatment Ladder in Dermatomyositis

SKIN DISEASE vs MUSCLE DISEASE
First Line vs Second Line vs Third Line

Photoprotection

A

First line for skin disease

29
Q

TABLE 62-4
Treatment Ladder in Dermatomyositis

SKIN DISEASE vs MUSCLE DISEASE
First Line vs Second Line vs Third Line

Topical steroids

A

First line for skin disease

30
Q

TABLE 62-4
Treatment Ladder in Dermatomyositis

SKIN DISEASE vs MUSCLE DISEASE
First Line vs Second Line vs Third Line

Hydroxychloroquine or Chloroquine

A

First line for skin disease

31
Q

TABLE 62-4
Treatment Ladder in Dermatomyositis

SKIN DISEASE vs MUSCLE DISEASE
First Line vs Second Line vs Third Line

Quinacrine

A

First line for skin disease

32
Q

TABLE 62-4
Treatment Ladder in Dermatomyositis

SKIN DISEASE vs MUSCLE DISEASE
First Line vs Second Line vs Third Line

Systemic corticosteroids

A

First line for muscle disease

33
Q

TABLE 62-4
Treatment Ladder in Dermatomyositis

SKIN DISEASE vs MUSCLE DISEASE
First Line vs Second Line vs Third Line

Methotrexate

A

Second line for skin and muscle disease

34
Q

TABLE 62-4
Treatment Ladder in Dermatomyositis

SKIN DISEASE vs MUSCLE DISEASE
First Line vs Second Line vs Third Line

Mycophenolate mofetil

A

Second line for skin and muscle disease

35
Q

TABLE 62-4
Treatment Ladder in Dermatomyositis

SKIN DISEASE vs MUSCLE DISEASE
First Line vs Second Line vs Third Line

Intravenous immune globulin

A

Second line for skin and muscle disease

36
Q

TABLE 62-4
Treatment Ladder in Dermatomyositis

SKIN DISEASE vs MUSCLE DISEASE
First Line vs Second Line vs Third Line

Azathioprine

A

Second line for skin and muscle disease

37
Q

TABLE 62-4
Treatment Ladder in Dermatomyositis

SKIN DISEASE vs MUSCLE DISEASE
First Line vs Second Line vs Third Line

Tofacitnib

A

Third line for skin disease

38
Q

TABLE 62-4
Treatment Ladder in Dermatomyositis

SKIN DISEASE vs MUSCLE DISEASE
First Line vs Second Line vs Third Line

Leflunomide

A

Third line for skin and muscle disease

39
Q

TABLE 62-4
Treatment Ladder in Dermatomyositis

SKIN DISEASE vs MUSCLE DISEASE
First Line vs Second Line vs Third Line

Dapsone

A

Third line for skin disease

40
Q

TABLE 62-4
Treatment Ladder in Dermatomyositis

SKIN DISEASE vs MUSCLE DISEASE
First Line vs Second Line vs Third Line

Thalidomide

A

Third line for skin disease

41
Q

TABLE 62-4
Treatment Ladder in Dermatomyositis

SKIN DISEASE vs MUSCLE DISEASE
First Line vs Second Line vs Third Line

Cyclosporine

A

Third line for skin and muscle disease

42
Q

TABLE 62-4
Treatment Ladder in Dermatomyositis

SKIN DISEASE vs MUSCLE DISEASE
First Line vs Second Line vs Third Line

Cyclophosphamide

A

Third line for skin and muscle disease

43
Q

TABLE 62-4
Treatment Ladder in Dermatomyositis

SKIN DISEASE vs MUSCLE DISEASE
First Line vs Second Line vs Third Line

Rituximab

A

Third line for muscle disease

Fitzpatrick Tables (55 decks)

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