3 - Vasculitis Flashcards
2 conditions which result in reddish and/or purplish change in skin color
- Vasculitis (the general disease we are studying)
- Pigmented purpuric lesions (the most common skin manifestation of vasculitis)
Vasculitis
- 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
Histological progression of vasculitis
- 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
Vasculitis changes
- **Changes are NOT confined just to skin, but are often a manifestation of systemic disease ***
- Joints, GI, heart, lung, kidney, CNS
Vasculitis lesions
- Lesions have predilection for leg –> Vasculitis loves the legs ***
- Very painful, bilateral and symmetrical
Progression of lesions
o Erythematous maculopapule o Urticarial papule o Palpable purpura, usually! o Hemorrhagic vesicle and bulla o Necrosis and ulceration
Purpura
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
Diascopy
testing for BLANCHING of the skin
How to do diascopy
- 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)
How do you classify vasculitis
Based on the size of the vessel if affects
Vasculitis that affects capillaries
o Leukocytoclastic vasculitis
o Henoch-Schoenlein purpura
Vasculitis that affects small arteries
o Granulomatous vasculitis
o Henoch-Schoenlein purpura
- The vast majority of vasculitis involves SMALL ARTERIES
- Along with capillaries, most likely to have palpable purpura
Vasculitis that affects medium arteries
PAN (polyarteritis nodosa)
Vasculitis that affects large arteries
o Giant cell arteritis
The most common skin manifestation of vasculitis is: o Necrosis o Palpable purpura o Macule o Ulceration o Cyst
Palpable purpura - doesn’t mean you won’t get the others, just not as common
Mnemonic for major etiologies of purpura
** KNOW THIS **
“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)
H = Henoch-Schonlein Purpura (“Hen”)
o AKA “IgA vasculitis”
o **Seen in children and young adults
o **Preceded by pharyngitis (usually strep throat)
o Affects bowels, kidneys, and joints
Diagnosis of Henoch-Schonlein Purpura
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
P = PAN (polyarteritis nodosa) (“Pain”)
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***
Diagnosis of PAN
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
W = Wegener’s Granulomatosis (“With”)
o Primarily affects lungs and kidneys
o Has both granulomas and vasculitis
o Cause nail fold infarcts and purpura
Diagnosis of Wegener’s
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
G = Giant cell arteritis (“Giant”)
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
Diagnosis of giant cell arteritis
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
