3: Vasculitis - Mahoney (6 Questions) Flashcards
vasculitis and pigmented purpuric lesions both result in…
some reddish and/or purplish change in skin color
define vasculitis
- Inflammation and necrosis of blood vessels caused by the precipitation of immune complexes in the vessel walls
- Seen with collagen vascular disease and cryoglobulinemia, as examples
- Changes not confined just to skin, but are often a manifestation of systemic disease
- Joints, GI, heart, lung, kidney, CNS
- Lesions have predilection for leg
histology of vasculitis
- Inflammatory infiltrate (PML’s)
- Leukocytoclasis (disintegration of PML’s)
- Extravasation of RBC’s
- Necrosis
- Hyalinization (degenerative process in tissue which results in clumping of acidophilic structures that appear glassy in appearance)
describe vasculitic lesion
very painful, bilateral, and symmetrical
progression of vasculitic lesions
erythematous maculopapule –> urticarial papule –> palpable/nonpalpable purpura –> hemorrhagic veiscle and bulla –> necrosis and ulceration
the _____ the vessel, the more common ulceration and necrosis becomes
common
what is diascopy?
If a skin lesion blanches when you apply pressure over it with a glass slide, it is composed of intact vessels that are inflammed
If the lesion persists under pressure, it is a skin pigmentation (e.g. purpura due to extrasavation of blood)
classification of blood vessels based on size of artery ... capillary small artery medium artery large artery
capillary-leukocytoclastic vasculitis (LCV) and Henoch-Schoenlein purpura
small artery- granulomatous vasculitis, Henoch-Schoenlein purpura
- Vast majority of vasculitis involves small arteries
- Along with capillaries, most likely to have palpable purpura
medium artery-PAN (polyarteritis nodosa)
large artery-giant cell arteritis
most common skin manifestation of vasculitis Necrosis Palpable purpura Macule Ulceration Cyst
palpable purpura
major etiologies of purpura
“The Sleepy Giant’s Hen Cried With Pain”
thrombocytopenia (and coagulopathies - non palpable) SLE Giant Cell arteritis Henoch-Schonlein purpura Cryoglobulinemia Wegener granulomatosis PAN
children and young adults
preceded by pharyngitis
Henoch-Schonlein Purpura
aka IgA vasculitis
henoch-schonelin purpura
diagnosis henoch-schonlein purpura
- biopsy
association with hepatitis B
PAN
- necrotizin inflammatin with common manifestation of nodules
diagnosis of PAN
confirmed by arterial aneurysms on aangiography
new onset diastolic blood pressure greater than 90 mm Hg
PAN
causes nail fold infarctts and purpura
wegener’s granulomatosis
- primarily affects lungs and kidnyes
- has both granulomas and vasculitis
C ANCA +
wegener’s granulomatosis
most common type of primary systemic vasculitis
giant cell arteritis
giant cell arteritis is associated with what disease
polymyalgia rheumatica manifested by shoulder and pelvic girdle pain
- age greater than 50
- localized headache of new onset
- sed rate greater than 60
- do biopsy
giant cell arteritis / polymyalgia rheumatica
– Cold precipitable proteins that migrate with gamma globulins on electrophoresis
— are immune complexes
associated with hepatitis C infection
cryoglobulinemia (three Cs on list)
only way to diagnose a vasculitis ***
must be a deep punch biopsy
lab work up cryoglobulinemia
Cyroglobulinemia
Hypocomplementemia
Hepatitis screen
lab work up SLE
antinuclear antibodies
anti-double stranded deoxyribonuclease (anti-dsDNA)
lab work up henoch-schonlein purpura
ASO titres (anti-strep )
Urinalysis
Serum creatinine
lab work up thrombocytopenia or coagulopathy
Platelets
PTT
PTT
lab work up PAN
- urinalysis
- creatinine
- hep screen
lab work up Wegener’s granulomatosis
- ANCA
- chest film
lab work up Giant cell arteritis
- EST
tx vascultiis
- high does steroids
- fyclophopamide
A patient exhibits purpuric lesions that are not palpable. Your first lab test ordered would be ESR Hepatitis Chest film platelet count ASO
platelet
variants of schamberg’s dx
pigmented purpuric dermatosis
descrone pigmented purpuric dermatosis
– Look like vasculitis (look purplish), but are not autoimmune diseases
– Many are variants of Schamberg’s Disease
asymptomatic (may itch)
– minute petechial lesions which begin on dorsum of feet, ankles and lower legs
– rarely atrophies
– normal clotting mechanisms and platelet function
cayenne pepper disease
pigmented purpuric dermatosis
tx pigmented purpuric dermatosis
Bioflavinoids (rutoside-50 mgs bid) and Vit C (500 mgs bid)
Purplish, mottled vascular pattern which is fish net-like
livedo reticularis
describe livedo reticularis
Purplish, mottled vascular pattern which is fish net-like May indicate systemic disease Usually just a local idiopathic reaction females, 25-40 y/o worse in winter
Spasm of cutaneous arteries followed by dilatation of subpapillary venous plexus
slowing of blood flow
thrombosis
ischemia outlines the areas between cones as violaceous reticulum
Cone is an areas of skin supplied by an arteriole
Idiopathic ivory white plaques stippled with telangiectasias and surrounded by erythema, petechia, or hyperpigmentation usually on legs and ankles of healthy women
atrophie blanche
Necrosis of epidermis and superficial dermis
purpura
infarcts heal
white plaques
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:
livedo reticularis
- palpable purpura in pts w/ neither thrombocytopenia nore coagulopathy
- arthritis/arthralgia
- abdominal pain
- renal dz w/ biopsy showing IgA nephropathy
henoch schonlein purpura
- tx with steroids
- most cases resolve spontaneously w/i 1 month
definitive diagnosis henoch schonlein purpura
skin biopsy
