Weedon Ch 8: VASCULOPATHIC Flashcards

Polyarteritis nodosa
Terms Definitions
Histology of Senile Purpura
- extravasated RBCs w/o inflammation
- solar elastosis
- thin epidermis with atrophy of collagen bundles
Warfarin Necrosis occurs when? assoc w/ what deficiency?
Occurs 2-5 days after warfarin is started;
Assoc w/ Protein C Deficiency
What factors does warfarin inhibit?
Vitamin K dependent factors (II, VII, IX, X) and protein C & S; get paradoxical coagulation due to short half-life of Protein C
Histology of Warfarin necrosis?
fibrin thrombi in vessels of deep dermis
Synonyms for Atrophie Blanche
Livedoid vasculopathy
Livedoid vasculitis
PURPLE (painful purpuric ulcers with reticular pattern of the lower extremities)
Clinical picture of Atrophie Blanche
middle age females, assoc w/ trhombogenic state
purple reticulated macules and punched out ulcers, heal with atrophic white stellate scars
Histology of Atrophic Blanche
Epidermis: necrotic, ulcerated or atrophic
Dermis: hyaline thrombi, dermal fibrosis/sclerosis, extravasated RBCs
Blood Vessels: Hyalinized vessels, sparse perivascular lymphocytes
DIF: immunoglobulins, complement and fibrin in blood vessels
Purpura Fulminans synonym?
Purpura gangrenosa;
Usu seen in children, variant of DIC
What is DIC?
An acquired systemic disorder;
Activation of coagulation pathway w/ consumption of clotting factors;
Labs: decreased platelets and fibrinogen, elevated PT, PTT and fibrin degradation products;
Histology in DIC?
fibrin thrombi, hemorrhage, necrosis of epidermis and sweat glands -- no inflammation
Differentiate the types of Cryoglobulinemia clinically
Type I - monoclonal IgM or IgG
Assoc with multiple myeloma, CLL, lymphoma, Waldenstrom's macroglobulinemia
Type II (mixed) and Type III (polyclonal): Assoc with infections (Hep C, EBV and Rheumatologic d/o like RA & SLE)
Differentiate Cryoglobulinemias histologically
Type I -- thombi, extravasated RBCs, sparse perivascular lymphocytes
Type II -- LCCV
Cholesterol Emboli Histology
cholesterol clefts and fibrin thromi
clinical: livedo reticularis & gangrene
Urticaria Histology
reticular dermal edema
sparse perivascular inflammation w/ eos
In Chronic Urticaria, the circulating Ab target with receptor?
high-affinity IgE receptor (FcεRIα)
Causes of LCCV?
Infections: Group A Strep, Mycobacterium tuberculosis
Drugs: PCN, Thiazides, sulfonamides, phenytoin, NSAIDS
Rheumatologic: RA, SLE
Histology of LCCV?
fibrinoid necrosis of vessel walls
leukocytoclasia
extravasated RBCs
DIF: granular IgG, IgM, complement in superficial blood vessels (IgA in HSP)
Biopsy: H&E, 16-36hours
DIF < 24hours
Histology Urticarial Vasculitis
LCCV w/ Eos
- Different from normal urticaria: Associated with burning sensation, lasts >24 hours (non-migrating), resolves with PIPA
- assoc with Rheum d/o or hypocomplementemia
What is Schnitzler's syndrome?
Urticarial vasculitis
Arthralgias(bone pain, hyperostosis), LAD, fever (increased ESR)
IgM monoclonal gammopathy (esp. Waldenstrom macroglobulinemia)
What is the tetrad of HSP?
palpable purpura, arthritis, abdominal pain, glomerulonephritis
Clinical Description of Erythema Elevatum Diutinum
- firm red to yellow-brown plaques on dorsal hands and feet, elbows & knees
- arthritis, pulmonary infiltrates, peripheral ulcerative keratitis
- assoc w/ RA, relapsing polychondritis, HIV, IgA monoclonal gammopathy
Histology of EED?
LCCV & dense infiltrate of neuts, fibrosis, +/- lipid deposits (cholesterol clefts); onion-skin like perivascular fibrosis; admixture of plasma cells, lymphocytes, and neuts
Granuloma faciale common in M or F?
Middle Age Males, no other assoc d/o
Pathology of Granuloma faciale
Grenz zone, mixed infiltrate
lots of eos
LCCV
Extravasated RBCs and hemosiderin
How do you distinguish EED from Granuloma faciale histologically?
Grenz zone and Eos
Clinical picture of polyarteritis nodosa
purpura
livedo reticularis
painful subcutaneous nodules
What dz is assoc with polyarteritis nodosa?
Assoc with Hep B
risk of Hairy Cell Leukemia
Histopath of polyarteritis nodosa?
LCCV of medium arteries in deep dermis or sq
intimal proliferation, thrombi, fibrosis
may see panniculitis near involved vessel
Three variants of superficial thrombophlebitis
Sclerosing lymphangitis of the penis - lesion near coronal sulcus from sex
Mondor's dz - 12% assoc with breast CA, cordlike lesion on chest
Trousseau's syndrome (migratory thrombophlebitis): paraneoplastic syndrome affecting upper extremities and trunk
Clinical manifestations of Wegener's
sinusitis & pulmonary dz
nephritis, oral ulcers, rhematoid-like nodules
c-ANCA+ (anti-PR3 positive)
Histopath of Wegener's
epidermis: necrotic/ulcerated
LCCV w/ neuts, lymphs, plasma cells
palisading granulomas of bv & dermis
thrombi, extravasated RBCs
Clincal manifestations of Churg Strauss
Initial Phase: Asthma
Second Phase: Fever & Eosinophilia & Gastroenteritis
Third phase: diffuse angiitis of different organs
P-ANCA+ (aka anti-myeloperoxidase) -- correlates with disease severity
Histopath of Churg Strauss
small vessel LCCV
dermal eos/flame figures
palisading granulomas w/in bv & surrounding dermis
List the histology & variants of Pigmented Purpuras
Histology: extravasated RBCs, perivascular lymphos, hemosiderin

Schamberg's disease
Majocchi's disease aka purpura annularis telangiectoides
Pigmented purpuric lichenoid dermatitis of Gougerot & Blum (lichenoid rxn instead of perivascular)
Eczemtidlike purpura of Doucas & Kapetanakis: scale & spongiosis
Lichen Aureus: solitary gold-colored macule or plaque
What are some dz assoc with Sweets Syndrome?
aka "acute febrile neutrophilic dermatosis"
Assoc: CML, arthritis, IBD, prior URI, and drugs (all-trans retinoic acid, G/GM-CSF)
Histopath for Sweets
Superficial dermal edema w/ streamers
Sea of Neutrophils
Leukocytoclasia w/o true vasculitis
What does Rheumatoid Neutrophilic Dermatosis like like?
Sweets, except it is assoc with RA
HLA assoc for Behcet's
HLA-Bw51
What antigen is being targeted in Behcet's?
HSP60 by autoreactive T-cells
Clinical manifestations of Behcets
oral & genital ulcers
uveitis
cutaneous pustules w/ pathergy
Histopath for Behcet's Dz?
epidermis w/ ulceration or pustule
diffuse dermal mixed inflam +/- vasculitis
Dz Assoc with Pyoderma gangrenosum?
IBD, myelogenous leukemia
What is PAPA Syndrome?
Pyogenic sterile Arthrtis, Pyoderma gangrenosum, Acne
Pathology of Pyoderma gangrenosum
Epidermal necrosis/ulceration
Pseudoepitherliomatous hyperplasia at edge
diffuse neutrophils
lymphocytic vasculitis at edge
Pathohistology of PLC
focal parakeratosis
spongiosis
basal layer liquefaction
perivascular CD4+ lymphos
extravasated RBCs
Pathohistology of PLEVA
aka Mucha-Habermann disease
P = Parakeratosis
L = Lichenoid infiltrate (CD8+)
E = Extravasated RBCs
V = V-shaped infiltrate
A = Apoptotic keratinocytes
Degos Disease Clinical picture
red macules on truck, heal with porcelain white scars ("footprints in the snow")
50% mortality from brain or bowel infarcts
Histopath for Degos Disease
atrophic epidermis
wedge-shaped dermal infarct
necrotic adnexal structures
dermal mucin early, sclerosis late
thrombosed arteriole in SubQ fat (difficult to find)
Eponym for Degos Disease
Malignant Atrophic Papulosis
or, Papulosis atrophicans maligna
In the group of fatal obliterative arteritis syndrome
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