Skin & Vascular Disorders Flashcards

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1
Q

Symptoms of polymyalgia rheumatica

A

Pain and stiffness in proximal muscles (eg, shoulders, hips), often with fever, malaise, weight loss
Does not cause muscular weakness

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2
Q

Epidemiology of polymyalgia rheumatica + association

A

More common in females > 50 years old; associated with giant cell (temporal) arteritis

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3
Q

Lab findings in polymyalgia rheumatica

A

Increased ESR & CRP, normal CK

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4
Q

Tx of polymyalgia rheumatica

A

Rapid response to low-dose glucocorticoids

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5
Q

Epidemiology of Fibromyalgia

A

Most common in females 20–50 years old

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6
Q

Define fibromyalgia

A

Chronic, widespread musculoskeletal pain associated with “tender points,” stiffness, paresthesias, poor sleep, fatigue, cognitive disturbance (“fibro fog”)

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7
Q

Lab findings of fibromyalgia

A

Normal inflammatory markers like ESR

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8
Q

Tx of fibromyalgia

A

Regular exercise, antidepressants (TCAs, SNRIs), neuropathic pain agents (eg, gabapentin)

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9
Q

Lab findings in non/specific polymyositis/dermatomyositis

A

Nonspecific: ⊕ ANA, increased CK
Specific: ⊕ anti-Jo-1 (histidyl-tRNA synthetase), ⊕ anti-SRP (signal recognition particle), ⊕ anti-Mi-2 (helicase)

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10
Q

Define polymyositis, involves what

A

Progressive symmetric proximal muscle weakness, characterized by endomysial inflammation with CD8+ T cells
Most often involves shoulders

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11
Q

Findings in dermatomyositis

A

Clinically similar to polymyositis, but also involves Gottron papules, photodistributed facial erythema (eg, heliotrope edema of the eyelids), “shawl and face” rash, mechanic’s hands (thickening, cracking, irregular “dirty” appearing marks due to hyperkeratosis of digital skin)

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12
Q

Dermatomyositis increases the risk of what

A

Occult malignancy

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13
Q

Inflammation in dermatomyositis

A

Perimysial inflammation and atrophy with CD4+ T cells

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14
Q

Define myositis ossificans

A

Heterotopic ossification involving skeletal muscle (eg, quadriceps)
Associated with blunt muscle trauma

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15
Q

Presentation of myositis ossificans

A

Painful soft tissue mass

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16
Q

Imaging and histology for myositis ossificans

A

Imaging: eggshell calcification.
Histology: metaplastic bone surrounding area of fibroblastic proliferation
Benign, but may be mistaken for sarcoma.

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17
Q

Name the large vessel vasculitis

A

Giant cell (temporal) arteritis & Takayasu arteritis

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18
Q

Epidemiology/presentation of giant cell arteritis

A

Females > 50 years old.
Unilateral headache, possible temporal artery tenderness, jaw claudication
May lead to irreversible blindness due to anterior ischemic optic neuropathy
Associated with polymyalgia rheumatica

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19
Q

Lab findings of giant cell arteritis

A

Most commonly affects branches of carotid artery
Focal granulomatous inflammation
Increase in ESR
IL-6 levels correlate with disease activity

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20
Q

Tx of giant cell arteritis

A

High-dose glucocorticoids prior to temporal artery biopsy to prevent blindness

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21
Q

Epidemiology/presentation of Takayasu arteritis

A

Usually Asian females < 40 years old. “Pulseless disease” (weak upper extremity pulses), fever, night sweats, arthritis, myalgias, skin nodules, ocular disturbances

22
Q

Lab findings of Takayasu

A

Granulomatous thickening and narrowing of aortic arch and proximal great vessels
Increased ESR

23
Q

Tx of Takayasu

A

Glucocorticoids

24
Q

What are the medium-vessel vasculitis

A

Buerger disease (thromboangiitis obliterans), Kawasaki disease, Polyarteritis nodosa

25
Q

Epidemiology/presentation of Buerger disease (thromboangiitis obliterans) and definition

A

Heavy tobacco smoking history, males < 40 years old
Intermittent claudication
May lead to gangrene, autoamputation of digits, superficial nodular phlebitis
Raynaud phenomenon is often present
Segmental thrombosing vasculitis with vein and nerve involvement

26
Q

Tx of Buerger disease (thromboangiitis obliterans)

A

Smoking cessation

27
Q

Epidemiology/presentation of Kawasaki disease

A

Usually Asian children < 4 years old.
Bilateral nonexudative bulbar Conjunctivitis, Rash (polymorphous = desquamating), Adenopathy (cervical), Strawberry tongue (oral mucositis), Hand-foot changes (edema, erythema), fever.
“CRASH and burn (fever) on a Kawasaki”

28
Q

What can occur as a result in Kawasaki disease

A

May develop coronary artery aneurysms ; thrombosis or rupture can cause death

29
Q

Tx of Kawasaki

A

IV immunoglobulins, aspirin

30
Q

Epidemiology/presentation of polyarteritis nodosa

A

Usually middle-aged males.
Hepatitis B seropositivity in 30% of patients
Fever, weight loss, malaise, headache
GI: abdominal pain, melena
Hypertension, neurologic dysfunction, cutaneous eruptions, renal damage

31
Q

Vessel involvement in polyarteritis nodosa and inflammation

A

Typically involves renal and visceral vessels, not pulmonary arteries
Different stages of transmural inflammation with fibrinoid necrosis

32
Q

Lab findings of polyarteritis nodosa

A

Innumerable renal microaneurysms and spasms on arteriogram (string of pearls appearance)

33
Q

Tx of polyarteritis nodosa

A

Glucocorticoids, cyclophosphamide

34
Q

What are the small vessel vasculitis

A

Behçet syndrome, Cutaneous small- vessel vasculitis, Eosinophilic granulomatosis with polyangiitis, Granulomatosis with polyangiitis, IgA vasculitis, microscopic polyangiitis, mixed cryoglobulinemia

35
Q

Presentation of eosinophilic
granulomatosis with polyangiitis

A

Asthma, sinusitis, skin nodules or purpura, peripheral neuropathy (eg, wrist/foot drop)
Can also involve heart, GI, kidneys (pauci-immune glomerulonephritis)

36
Q

Inflammation in eosinophilic granulomatosis with polyangiitis + lab findings

A

Granulomatous, necrotizing vasculitis with eosinophilia
MPO-ANCA/p-ANCA, Increased IgE lvl

37
Q

Presentation of granulomatosis with polyangiitis

A

Upper respiratory tract: perforation of nasal septum, chronic sinusitis, otitis media, mastoiditis
Lower respiratory tract: hemoptysis, cough, dyspnea
Renal: hematuria, red cell casts

38
Q

Inflammation in granulomatosis with polyangiitis

A

Triad:
Focal necrotizing vasculitis
Necrotizing granulomas in lung and upper airway
Necrotizing glomerulonephritis

39
Q

Lab findings in granulomatosis with polyangiitis

A

PR3-ANCA/c-ANCA (anti-proteinase 3) CXR: large nodular densities

40
Q

Tx of granulomatosis with polyangiitis

A

Glucocorticoids in combination with
rituximab/cyclophosphamide

41
Q

Epidemiology/Presentation of IgA vasculitis

A

Most common childhood systemic vasculitis
Often follows URI
Classic triad: hinge pain (arthralgias), stomach pain (abdominal pain associated with intussusception), palpable purpura on buttocks/legs

42
Q

Definition of IgA vasculitis

A

Vasculitis 2° to IgA immune complex deposition
Associated with IgA nephropathy (Berger disease)

43
Q

Tx of IgA vasculitis

A

Supportive care, possibly glucocorticoids

44
Q

Presentation of microscopic polyangiitis

A

Necrotizing vasculitis commonly involving lung, kidneys, and skin with pauci-immune glomerulonephritis and palpable purpura
Presentation similar to granulomatosis with polyangiitis but without nasopharyngeal involvement

45
Q

Lab findings of microscopic polyangiitis

A

No granulomas
MPO-ANCA/p-ANCA J (anti-myeloperoxidase)

46
Q

Tx of microscopic polyangiitis

A

Cyclophosphamide, glucocorticoids

47
Q

Presentation of mixed cryoglobulinemia

A

Often due to viral infections, especially HCV
Triad of palpable purpura, weakness, arthralgias
May also have peripheral neuropathy and renal disease (eg, glomerulonephritis)

48
Q

Definition of mixed cryoglobulinemia

A

Cryoglobulins are immunoglobulins that precipitate in the Cold
Vasculitis due to mixed IgG & IgM immune complex deposition

49
Q

Epidemiology/presentation of Bechet syndrome

A

People of Turkish and Eastern Mediterranean descent
Recurrent aphthous ulcers, genital ulcerations, uveitis, erythema nodosum
Can be precipitated by HSV or parvovirus
Flares last 1–4 weeks

50
Q

Causes and gene association in Bechet

A

Immune complex vasculitis
Associated with HLA-B51

51
Q

Define cutaneous small-vessel vasculitis

A

Occurs 7–10 days after certain medications (penicillins, cephalosporins, sulfonamides, phenytoin, allopurinol) or infections (eg, HCV, HIV)
Palpable purpura, no visceral involvement

52
Q

Lab findings and late involvement in cutaneous small-vessel vasculitis

A

Immune complex-mediated leukocytoclastic vasculitis
Late involvement indicates systemic vasculitis.