Skin & Vascular Disorders Flashcards

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
Epidemiology/presentation of Buerger disease (thromboangiitis obliterans) and definition
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
Tx of Buerger disease (thromboangiitis obliterans)
Smoking cessation
27
Epidemiology/presentation of Kawasaki disease
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
What can occur as a result in Kawasaki disease
May develop coronary artery aneurysms ; thrombosis or rupture can cause death
29
Tx of Kawasaki
IV immunoglobulins, aspirin
30
Epidemiology/presentation of polyarteritis nodosa
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
Vessel involvement in polyarteritis nodosa and inflammation
Typically involves renal and visceral vessels, not pulmonary arteries Different stages of transmural inflammation with fibrinoid necrosis
32
Lab findings of polyarteritis nodosa
Innumerable renal microaneurysms and spasms on arteriogram (string of pearls appearance)
33
Tx of polyarteritis nodosa
Glucocorticoids, cyclophosphamide
34
What are the small vessel vasculitis
Behçet syndrome, Cutaneous small- vessel vasculitis, Eosinophilic granulomatosis with polyangiitis, Granulomatosis with polyangiitis, IgA vasculitis, microscopic polyangiitis, mixed cryoglobulinemia
35
Presentation of eosinophilic granulomatosis with polyangiitis
Asthma, sinusitis, skin nodules or purpura, peripheral neuropathy (eg, wrist/foot drop) Can also involve heart, GI, kidneys (pauci-immune glomerulonephritis)
36
Inflammation in eosinophilic granulomatosis with polyangiitis + lab findings
Granulomatous, necrotizing vasculitis with eosinophilia MPO-ANCA/p-ANCA, Increased IgE lvl
37
Presentation of granulomatosis with polyangiitis
Upper respiratory tract: perforation of nasal septum, chronic sinusitis, otitis media, mastoiditis Lower respiratory tract: hemoptysis, cough, dyspnea Renal: hematuria, red cell casts
38
Inflammation in granulomatosis with polyangiitis
Triad: Focal necrotizing vasculitis Necrotizing granulomas in lung and upper airway Necrotizing glomerulonephritis
39
Lab findings in granulomatosis with polyangiitis
PR3-ANCA/c-ANCA (anti-proteinase 3) CXR: large nodular densities
40
Tx of granulomatosis with polyangiitis
Glucocorticoids in combination with rituximab/cyclophosphamide
41
Epidemiology/Presentation of IgA vasculitis
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
Definition of IgA vasculitis
Vasculitis 2° to IgA immune complex deposition Associated with IgA nephropathy (Berger disease)
43
Tx of IgA vasculitis
Supportive care, possibly glucocorticoids
44
Presentation of microscopic polyangiitis
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
Lab findings of microscopic polyangiitis
No granulomas MPO-ANCA/p-ANCA J (anti-myeloperoxidase)
46
Tx of microscopic polyangiitis
Cyclophosphamide, glucocorticoids
47
Presentation of mixed cryoglobulinemia
Often due to viral infections, especially HCV Triad of palpable purpura, weakness, arthralgias May also have peripheral neuropathy and renal disease (eg, glomerulonephritis)
48
Definition of mixed cryoglobulinemia
Cryoglobulins are immunoglobulins that precipitate in the Cold Vasculitis due to mixed IgG & IgM immune complex deposition
49
Epidemiology/presentation of Bechet syndrome
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
Causes and gene association in Bechet
Immune complex vasculitis Associated with HLA-B51
51
Define cutaneous small-vessel vasculitis
Occurs 7–10 days after certain medications (penicillins, cephalosporins, sulfonamides, phenytoin, allopurinol) or infections (eg, HCV, HIV) Palpable purpura, no visceral involvement
52
Lab findings and late involvement in cutaneous small-vessel vasculitis
Immune complex-mediated leukocytoclastic vasculitis Late involvement indicates systemic vasculitis.