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a;lskdjf > 3 - Vasculitis > Flashcards

3 - Vasculitis Flashcards

1
Q

2 conditions which result in reddish and/or purplish change in skin color

A
  • Vasculitis (the general disease we are studying)

- Pigmented purpuric lesions (the most common skin manifestation of vasculitis)

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2
Q

Vasculitis

A
  • Inflammation and necrosis of blood vessels caused by the precipitation of immune complexes in the vessel walls
  • Vasculitis is seen with collagen vascular disease and cryoglobulinemia, for example
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3
Q

Histological progression of vasculitis

A
  • Inflammatory infiltrate (PMLs)
  • Leukocytoclasis (disintegration of PMLs)
  • Extravasation of RBCs
  • Necrosis
  • Hyalinization (degenerative process in tissue which results in clumping of acidophilic structures that appear glassy in appearance
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4
Q

Vasculitis changes

A
  • **Changes are NOT confined just to skin, but are often a manifestation of systemic disease ***
  • Joints, GI, heart, lung, kidney, CNS
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5
Q

Vasculitis lesions

A
  • Lesions have predilection for leg –> Vasculitis loves the legs ***
  • Very painful, bilateral and symmetrical
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6
Q

Progression of lesions

A 
o	Erythematous maculopapule
o	Urticarial papule
o	Palpable purpura, usually!
o	Hemorrhagic vesicle and bulla
o	Necrosis and ulceration
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7
Q

Purpura

A

o Realize that the purpura can be palpable or non-palpable!!!!!!!!!!! There is ALWAYS purpura and USUALLY palpable, but NOT always***
o The larger the vessel, the more common ulceration and necrosis becomes

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8
Q

Diascopy

A

testing for BLANCHING of the skin

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9
Q

How to do diascopy

A
  • Apply moderate pressure over lesion with a glass slide, look for the color reaction of the skin
  • Blanches (turns white)  Lesion is composed of intact vessels that are inflamed
  • Persists (stays purplish)  Lesion is a skin pigmentation (e.g. purpura – extravasation of blood)
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10
Q

How do you classify vasculitis

A

Based on the size of the vessel if affects

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11
Q

Vasculitis that affects capillaries

A

o Leukocytoclastic vasculitis

o Henoch-Schoenlein purpura

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12
Q

Vasculitis that affects small arteries

A

o Granulomatous vasculitis
o Henoch-Schoenlein purpura

  • The vast majority of vasculitis involves SMALL ARTERIES
  • Along with capillaries, most likely to have palpable purpura
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13
Q

Vasculitis that affects medium arteries

A

PAN (polyarteritis nodosa)

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14
Q

Vasculitis that affects large arteries

A

o Giant cell arteritis

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15
Q 
The most common skin manifestation of vasculitis is:
o	Necrosis
o	Palpable purpura 
o	Macule 
o	Ulceration 
o	Cyst
A

Palpable purpura - doesn’t mean you won’t get the others, just not as common

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16
Q

Mnemonic for major etiologies of purpura

** KNOW THIS **

A

“The Sleepy Giant’s Hen Cried With Pain”
o T = thrombocytopenia (no additional notes below)
o S = SLE (lupus) (no additional notes below)
o G = Giant cell arteritis
o H = Henoch-Schonlein purpura
o C = Cryoglobulinemia
o W = Wegener granulomatosis
o P = PAN (polyarteritis nodosa)

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17
Q

H = Henoch-Schonlein Purpura (“Hen”)

A

o AKA “IgA vasculitis”
o **Seen in children and young adults
o **Preceded by pharyngitis (usually strep throat)
o Affects bowels, kidneys, and joints

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18
Q

Diagnosis of Henoch-Schonlein Purpura

A

o Palpable purpura in patients with neither thrombocytopenia or coagulopathy
o Arthritis or arthralgia
o Abdominal pain
o Renal disease
o BIOPSY  definitive diagnosis is by biopsy

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19
Q

P = PAN (polyarteritis nodosa) (“Pain”)

A

o Necrotizing inflammation with common manifestation of nodules
o Leads to ischemia or infarction of gut, heart, kidney
o Association with HEPATITIS B  When you see PAN, think Hep B***

20
Q

Diagnosis of PAN

A

o Arterial aneurysm seen on angiography of renal, hepatic, splanchnic/splenic circulations
o Unexplained weight loss more than 4 kg
o Livedo reticularis
o Testicular pain or tenderness
o Myalgias (excluding that of the shoulder and hip girdle), weakness or polyneuropathy
o Mononeuropathy or polyneuropathy
o New onset diastolic blood pressure >90 mmHg
o Elevated levels of creatinine
o Evidence of hepatitis B virus infection
o Arteriographic abnormalities not resulting from non-inflammatory disease processes
o BIOPSY  definitive diagnosis by biopsy

21
Q

W = Wegener’s Granulomatosis (“With”)

A

o Primarily affects lungs and kidneys
o Has both granulomas and vasculitis
o Cause nail fold infarcts and purpura

22
Q

Diagnosis of Wegener’s

A

o Nasal or oral inflammation
 Painful/painless oral ulcers or purulent/bloody nasal discharge
o Abnormal chest radiograph showing nodules, fixed infiltrates or cavities
o Abnormal urinary sedimentation (microscopic hematuria or red cell casts
o Antineutrophil cytoplasmic antibodies (ANCA)
o BIOPSY  definitive diagnosis by biopsy

23
Q

G = Giant cell arteritis (“Giant”)

A

o Most common type of primary systemic vasculitis
o Often associated with polymyalgia rheumatica which is manifested by shoulder and pelvic girdle pain
o Typically age 50+ at the time of disease onset

24
Q

Diagnosis of giant cell arteritis

A

o Localized headache during new onset
o Tenderness or decreased pulse of the temporal artery
o Erythrocyte sedimentation rate (ESR) greater than 50 mm/h (Westergren)
o BIOPSY  definitive diagnosis by biopsy

25
Q

C = Cryoglobulinemia (“Cries”)

A

o Cold precipitable proteins that migrate with gamma globulins on electrophoresis
o Are immune complexes
o Associated with hepatitis C infection  C for Hep C infection

26
Q

Diagnosis of cryoglobulinemia

A 
o	Arthralgias or arthritis 
o	Renal disease
o	Vasculitic erythematous skin lesions that are typically located on the lower extremities 
o	Cryoglobulinemia 
o	Hypocomplementemia 
o	Sensorimotor peripheral neuropathy
27
Q

Type of biopsy for vasculitis diagnosis

A
  • Diagnosis of vasculitis  **Biopsy is the ONLY way to diagnose vasculitis **
  • NEED to have a deep punch biopsy, NOT just regular depth (like what you would do for a mole)
28
Q

Workup for cryoglobulinemia

A

The 3 C’s:

  • Cryoglobulinemia
  • HypoComplementemia
  • Hepatitis screen (Hep C)
29
Q

Workup for SLE

A
  • Antinuclear antibodies (for lupus infection)

- Anti-double stranded deoxyribonuclease (anti-dsDNA) – more specific test – confirmation test

30
Q

Workup for Henoch-Schonlein purpura

A
  • ASO titres (look for previous strep infection)
  • Urinalysis (kidney involvement common)
  • Serum creatinine (kidney involvement common
    o NOTE  all of these can have kidney involvement, so do urinalysis and serum creatinine for all of them, but it is MORE common in Henoch-Schonlein (but not enough to make it unique)
31
Q

Thrombocytopenia or coagulopathy workup

A
  • Platelets

- PT, PTT

32
Q

Workup for PAN

A
  • Urinalysis
  • Creatinine
  • Hepatitis screen  Hepatitis B
33
Q

Workup for Wegener’s granulomatosis

A
  • ANCA (associated with ANCA  UNIQUE to Wegener’s)

- Chest film (loves the lung)

34
Q

Workup for giant cell arteritis

A

ESR

35
Q

Treatment of vasculitis

A
  • NOTE  you will NEED to identify the underlying cause for specific treatment
  • Common treatment for ALL vasculitis: (usually treated by rheumatology)
    o High dose steroids (suppress the intense inflammation)
    o Cyclophosmamide (possibly)
36
Q 
A patient exhibits purpuric lesions that are not palpable. Your first lab test ordered would be:
o	ESR
o	Hepatitis screen 
o	Chest film 
o	Platelet count 
o	ASO
A

4, platelet count because the most common non-palpable lesion is thrombocytopenia or coagulopathy

37
Q

Pigmented purpuric dermatosis

A

o Look like vasculitis (looks purplish), but are NOT autoimmune diseases
o Many are variants of Schamberg’s disease
- Benign skin lesion due to fragile vessels, common in diabetics
- Called “Cayenne Pepper Disease” due to resemblance to spilled cayenne pepper
- If you see this, do NOT think vasculitis, think Schamberg’s disease (benign)
o Asymptomatic (may itch)
- If wondering if it is benign Schamberg’s disease or not, ask if there are any other systemic manifestations (i.e. joint pain, edema, etc.)
o Minute petechial lesions (which begin on dorsum of feet, ankles and lower legs
o Rarely atrophies
o Normal clotting mechanisms and platelet function
o Also includes - livedo reticularis and atrophie blanche

38
Q

Etiology of pigmented purpuric dermatosis

A

o May be due to chronic irritant

o May be associated with stasis dermatitis

39
Q

Treatment of pigmented purpuric dermatosis

A

o Itching –> topical steroids
o Bioflavinoids (rutoside – 50 mgs BID)
o Vitamin C (500 mgs BID)

40
Q

Livedo reticularis

A

o Purplish, mottled vascular pattern which is fish net-like
o May indicate systemic disease
o Usually just a local idiopathic reaction
o Females 25-40 y/o
o Worse in WINTER

41
Q

Symptoms of livedo reticularis

A

o Spasm of cutaneous arteries followed by dilation of subpapillary venous plexus
o Slowing of blood
o Thrombosis
o Ischemia outlines areas between cones as violaceous reticulum –> Cone is an area of skin supplied by an arteriole

42
Q

Atrophie blanche

A

o Idiopathic ivory white plaques stippled with telangiectasias and surrounded by erythema, petechia or hyperpigmentation usually on legs and ankles of healthy women

43
Q

Etiology of atrophie blanche

A

o Necrosis of epidermis and superficial dermis
o Purpura
o Infarcts heal
o White plaques

44
Q 
You notice purple circular lesions on the feet and legs of a patient that are not palpable. The patient relates always having cold feet. The diagnosis is:
o	Schamberg’s disease
o	PAN 
o	Livedo reticularis
o	Raynaud’s disease
o	Likely a systemic disease
A

Livedo reticularis

45
Q

Case study

A
  • 30 year old male patient presents with painful, red, bleeding around all toe nails
  • All ten toe nails were removed but pain and bleeding continued – abnormal reaction
  • History revealed cold feet, physical revealed good pulses – should raise a red flag for Raynaud’s
  • Raynaud’s could be a sign of a systemic disease, most likely collagen vascular disease
  • Needed to ask more questions - positive for sores in mouth and penis, rashes that don’t go away, vision changes, joint pain in hands, toes, ankles and feet
  • Determined it is a connective tissue disorder or vasculitis
  • Referred to rheumatology, Basset’s disease was the diagnosis (?)
46
Q

Focus on

A
  • Signs and symptoms with each condition (“sleepy giants” mnemonic)
  • Changes that occur with each condition
  • Progression of lesions for each condition
  • Tests to run for each condition

a;lskdjf (11 decks)

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