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49 Cards in this Set
- Front
- Back
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Histology of Senile Purpura
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- extravasated RBCs w/o inflammation
- solar elastosis - thin epidermis with atrophy of collagen bundles |
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Warfarin Necrosis occurs when? assoc w/ what deficiency?
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Occurs 2-5 days after warfarin is started;
Assoc w/ Protein C Deficiency |
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What factors does warfarin inhibit?
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Vitamin K dependent factors (II, VII, IX, X) and protein C & S; get paradoxical coagulation due to short half-life of Protein C
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Histology of Warfarin necrosis?
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fibrin thrombi in vessels of deep dermis
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Synonyms for Atrophie Blanche
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Livedoid vasculopathy
Livedoid vasculitis PURPLE (painful purpuric ulcers with reticular pattern of the lower extremities) |
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Clinical picture of Atrophie Blanche
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middle age females, assoc w/ trhombogenic state
purple reticulated macules and punched out ulcers, heal with atrophic white stellate scars |
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Histology of Atrophic Blanche
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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 |
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Purpura Fulminans synonym?
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Purpura gangrenosa;
Usu seen in children, variant of DIC |
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What is DIC?
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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; |
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Histology in DIC?
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fibrin thrombi, hemorrhage, necrosis of epidermis and sweat glands -- no inflammation
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Differentiate the types of Cryoglobulinemia clinically
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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) |
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Differentiate Cryoglobulinemias histologically
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Type I -- thombi, extravasated RBCs, sparse perivascular lymphocytes
Type II -- LCCV |
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Cholesterol Emboli Histology
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cholesterol clefts and fibrin thromi
clinical: livedo reticularis & gangrene |
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Urticaria Histology
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reticular dermal edema
sparse perivascular inflammation w/ eos |
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In Chronic Urticaria, the circulating Ab target with receptor?
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high-affinity IgE receptor (FcεRIα)
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Causes of LCCV?
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Infections: Group A Strep, Mycobacterium tuberculosis
Drugs: PCN, Thiazides, sulfonamides, phenytoin, NSAIDS Rheumatologic: RA, SLE |
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Histology of LCCV?
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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 |
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Histology Urticarial Vasculitis
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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 |
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What is Schnitzler's syndrome?
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Urticarial vasculitis
Arthralgias(bone pain, hyperostosis), LAD, fever (increased ESR) IgM monoclonal gammopathy (esp. Waldenstrom macroglobulinemia) |
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What is the tetrad of HSP?
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palpable purpura, arthritis, abdominal pain, glomerulonephritis
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Clinical Description of Erythema Elevatum Diutinum
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- 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 |
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Histology of EED?
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LCCV & dense infiltrate of neuts, fibrosis, +/- lipid deposits (cholesterol clefts); onion-skin like perivascular fibrosis; admixture of plasma cells, lymphocytes, and neuts
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Granuloma faciale common in M or F?
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Middle Age Males, no other assoc d/o
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Pathology of Granuloma faciale
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Grenz zone, mixed infiltrate
lots of eos LCCV Extravasated RBCs and hemosiderin |
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How do you distinguish EED from Granuloma faciale histologically?
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Grenz zone and Eos
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Clinical picture of polyarteritis nodosa
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purpura
livedo reticularis painful subcutaneous nodules |
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What dz is assoc with polyarteritis nodosa?
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Assoc with Hep B
risk of Hairy Cell Leukemia |
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Histopath of polyarteritis nodosa?
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LCCV of medium arteries in deep dermis or sq
intimal proliferation, thrombi, fibrosis may see panniculitis near involved vessel |
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Three variants of superficial thrombophlebitis
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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 |
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Clinical manifestations of Wegener's
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sinusitis & pulmonary dz
nephritis, oral ulcers, rhematoid-like nodules c-ANCA+ (anti-PR3 positive) |
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Histopath of Wegener's
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epidermis: necrotic/ulcerated
LCCV w/ neuts, lymphs, plasma cells palisading granulomas of bv & dermis thrombi, extravasated RBCs |
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Clincal manifestations of Churg Strauss
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Initial Phase: Asthma
Second Phase: Fever & Eosinophilia & Gastroenteritis Third phase: diffuse angiitis of different organs P-ANCA+ (aka anti-myeloperoxidase) -- correlates with disease severity |
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Histopath of Churg Strauss
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small vessel LCCV
dermal eos/flame figures palisading granulomas w/in bv & surrounding dermis |
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List the histology & variants of Pigmented Purpuras
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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 |
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What are some dz assoc with Sweets Syndrome?
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aka "acute febrile neutrophilic dermatosis"
Assoc: CML, arthritis, IBD, prior URI, and drugs (all-trans retinoic acid, G/GM-CSF) |
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Histopath for Sweets
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Superficial dermal edema w/ streamers
Sea of Neutrophils Leukocytoclasia w/o true vasculitis |
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What does Rheumatoid Neutrophilic Dermatosis like like?
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Sweets, except it is assoc with RA
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HLA assoc for Behcet's
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HLA-Bw51
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What antigen is being targeted in Behcet's?
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HSP60 by autoreactive T-cells
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Clinical manifestations of Behcets
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oral & genital ulcers
uveitis cutaneous pustules w/ pathergy |
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Histopath for Behcet's Dz?
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epidermis w/ ulceration or pustule
diffuse dermal mixed inflam +/- vasculitis |
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Dz Assoc with Pyoderma gangrenosum?
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IBD, myelogenous leukemia
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What is PAPA Syndrome?
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Pyogenic sterile Arthrtis, Pyoderma gangrenosum, Acne
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Pathology of Pyoderma gangrenosum
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Epidermal necrosis/ulceration
Pseudoepitherliomatous hyperplasia at edge diffuse neutrophils lymphocytic vasculitis at edge |
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Pathohistology of PLC
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focal parakeratosis
spongiosis basal layer liquefaction perivascular CD4+ lymphos extravasated RBCs |
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Pathohistology of PLEVA
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aka Mucha-Habermann disease
P = Parakeratosis L = Lichenoid infiltrate (CD8+) E = Extravasated RBCs V = V-shaped infiltrate A = Apoptotic keratinocytes |
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Degos Disease Clinical picture
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red macules on truck, heal with porcelain white scars ("footprints in the snow")
50% mortality from brain or bowel infarcts |
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Histopath for Degos Disease
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atrophic epidermis
wedge-shaped dermal infarct necrotic adnexal structures dermal mucin early, sclerosis late thrombosed arteriole in SubQ fat (difficult to find) |
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Eponym for Degos Disease
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Malignant Atrophic Papulosis
or, Papulosis atrophicans maligna In the group of fatal obliterative arteritis syndrome |
