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WILLIAM'S HEMATOLOGY > 121 The Vascular Purpuras > Flashcards

121 The Vascular Purpuras Flashcards

1
Q

Visible hemorrhage into mucous membranes or skin, which corresponds to extravasation of red blood cells around small dermal vessels and chronic hemosiderin deposition

A

Purpura

Do not blanch completely upon compression

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2
Q

Blanching is commonly tested by compression of skin lesions with a glass slide, referred to as

A

Diascopy

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3
Q

Lesions that mimic purpura with incomplete blanching upon diascopy but are not purpura because no hemorrhage has occurred

A

Erythema

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4
Q

Small, focal areas of hemorrhage (≤4 mm)

A

Petechiae

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5
Q

Larger lesions (>4 mm, <1 cm)

A

Purpura

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6
Q

Larger lesions (≥1 cm)

A

Ecchymosis

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7
Q

Vascular anomalies that blanch with pressure

A

Telangiectasia

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8
Q

Presence of cold-insoluble immunoglobulins in plasma

A

Cryoglobulinemia

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9
Q

Types of cryoglobulinemia

A
  • Type I: Monoclonal IgG, IgM, or IgA
  • Type II (mixed): Complexes composed of polyclonal IgG with monoclonal immunoglobulins, typically IgM with anti-IgG specificity
  • Type III: Polyclonal IgG and IgM complexes
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10
Q

Precipitates of immunoglobulin light chains that form crystalline deposits in the skin causing hemorrhagic palpable purpura

A

Light-chain vasculopathy

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11
Q

Form of serum dysproteinemia characterized by formation of an abnormal cold-precipitable fibrinogen

A

Cryofibrinogenemia

Cutaneous manifestations of cryofibrinogenemia

* Cyanosis
* Erythema
* Raynaud phenomenon
* Palpable purpura of the nose, ears, and distal extremities

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12
Q

Cutaneous reactions to heparin administration including purpuric plaques with cutaneous ulceration or necrosis

A

Heparin necrosis

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13
Q

Characteristic of Warfarin necrosis

The new direct-acting oral anticoagulants are not associated with skin necrosis

A

Painful erythematous plaques and nodules that can rapidly become hemorrhagic and necrotic, leading to large areas of infarct with black eschar formation and subsequent skin sloughing

The onset is sudden after 2 to 14 days of drug therapy

Women are more commonly affected, and lesions most often involve thighs, buttocks, or breasts.

Coumarin necrosis is more likely to occur in patients with protein C deficiency and patients with heparin-induced thrombocytopenia.

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14
Q

Causes of warfarin necrosis

A

Rapid decrease of vitamin K-dependent coagulation factors of relatively short half-life, such as proteins C and S, while longer-lasting coagulation factors are not yet decreased, resulting in a net procoagulant state

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15
Q

Congenital and acquired deficiencies in proteins C and S

A
  • Can lead to palpable necrotic purpura and ecchymosis
  • Erythematous purpuric lesions associated with homozygous protein C deficiency can develop within hours of birth and can rapidly progress to hemorrhagic necrosis
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16
Q

Causes of Acquired deficiencies of protein C

A
  • Autoantibodies to protein C
  • Antibiotics administration
  • Septic shock
  • HIV
  • Liver disease
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17
Q

Causes of Acquired deficiencies of protein S

A
  • After varicella infection: generation of antiprotein S immunoglobulins
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18
Q

Treatment for protein C deficiency

A
  1. Protein repletion with fresh-frozen plasma or protein C concentrate as initial treatment to help clear both cutaneous lesions and venous occlusion
  2. Lifelong anticoagulant treatment is used to prevent recurrence
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19
Q

Skin manifestations of Antiphospholipid Syndrome

Presence of_________________________ is frequently the presenting symptom of APS, most commonly when the syndrome is secondary to SLE, and its presence commonly precedes vascular events

A

Livedo reticularis

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20
Q

A chronic recurrent thrombo-occlusive disorder characterized by the initial development of erythematous purpuric lesions with telangiectasis and peripheral petechiae, and lower-extremity ulcerations

Most commonly arising without associated cause, it is associated with polyarteritis nodosa, APS, and SLE

A

Livedoid Vasculitis

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21
Q

A term that refers to the appearance of ivory-white stellate scars commonly surrounded by hyperpigmented areas and telangiectasia

These lesions appear to be caused by small-vessel fibrin thrombi in the middle and lower dermis as a result of a procoagulant tendency

A

Atrophie blanche

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22
Q

Also known as atheroemboli, they are responsible for a syndrome characterized by lower extremity pain and livedo reticularis with preservation of peripheral pulses

Other common cutaneous findings include gangrene, purpura, ulcerations, cyanosis, and nodules

A

Cholesterol Crystal Emboli

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23
Q

Are the most common source of cholesterol emboli

A

Atherosclerotic lesions in the descending aorta

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24
Q

A thrombo-occlusive disorder involving formation of cutaneous, subcutaneous, and vascular calcifications

Most commonly seen in patients with end-stage renal disease, classically caused by the development of secondary hyperparathyroidism

Present initially as reddish-purple plaques, evolving to tender, gangrenous ulcers or reticular hemorrhagic necrosis

A

Calciphylaxis

  • Approximately 4% of hemodialysis-dependent patients have calciphylaxis
  • Survival is less than 50% at 5 years after diagnosis
  • Other etiologies include primary hyperparathyroidism, malignancy, alcoholic liver disease, and collagen vascular disorders
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25
Q

Acral purpuric lesions secondary to emboli arise from left atrial myxomas or right atrial clots through paradoxical embolization

A

Emboli from Intracardiac Thrombi

26
Q

Purpuric lesions are not uncommon after arthropod bites

Give characteristics of different arthropod bites

A

Bites from bed bugs, Cimex lectularius: localized purpuric macules or papules

Bites from kissing bugs, Reduviidae: urticaria with hemorrhagic bulla

Envenomation from a brown recluse spider, Loxosceles reclusa: purpuric necrosis with surrounding erythema evolving to ulcer formation

27
Q

May accompany infections with Pseudomonas spp, Klebsiella spp, Aeromonas hydrophilia, or Escherichia coli in patients with severe granulocytopenia or immune compromise

A

Ecthyma gangrenosum

28
Q

An idiopathic inflammatory skin condition characterized by early follicular erythematous papules and pustules, or tender, fluctuant nodules with surrounding erythema that spread peripherally and ulcerate, surrounded by a violaceous rim

A

Pyoderma Gangrenosum

In 50% of cases, there is an associated disorder, such as inflammatory bowel disorders (classically ulcerative colitis), arthritis, hematologic disorders, and solid tumors

29
Q

Four main clinical variants of Pyoderma Gangrenosum

A
  • Ulcerative
  • Pustular
  • Bullous
  • Vegetative
30
Q

Histopathologic finding of Pyoderma Gangrenosum

A

Sterile abscess with central necrotizing neutrophilic infiltration and a surrounding perivascular and intramural lymphocytic infiltration

31
Q

Treatment of Pyoderma Gangrenosum

A

Wound care and immunosuppressants, such as glucocorticoids, cyclosporine, dapsone, azathioprine, and infliximab

32
Q

Also referred to as acute, febrile neutrophilic dermatosis, it is characterized by the acute manifestation of painful erythematous and violaceous papules, nodules, and plaques accompanied by fever and elevated neutrophil count

A

Sweet Syndrome

  • These papules, which most commonly appear on the face, neck, and upper extremities, present a central yellowish discoloration and tend to coalesce, forming well-circumscribed, irregularly bordered plaques
  • Other organs can be involved, including the CNS, kidneys, lungs, and bones
  • Classically more prominent in middle-aged women, this syndrome associates a complex cytokine dysregulation
  • Other manifestations include respiratory and urinary infections and autoimmune disorders (including rheumatoid arthritis, SLE, and inflammatory bowel disease)
33
Q

Treatment of Sweet Syndrome

A

Systemic glucocorticoid

34
Q

An inflammatory disorder that affects multiple organ systems

Clinical features include chronic and relapsing cutaneous manifestations, such as palpable purpura, infiltrative erythema, and papulopustular lesions, as well as oral mucosal and genital ulcers, arthralgias, and gastrointestinal and CNS involvement

Histologic features include leukocytoclastic or lymphocytic vasculitis

A

Behçet Disease
(Variable-Vessel Vasculitis)

35
Q

Genetic studies show an association between Behçet disease and human leukocyte antigen ______

A

Human leukocyte antigen B51

36
Q

Reflects the clinical manifestations of immune complex formation and deposition

Cutaneous lesions such as urticarial and morbilliform eruptions predominate, although palpable purpura and erythema multiforme can also be encountered

A

Serum Sickness

37
Q

A predominantly pediatric vasculitic syndrome characterized by the acute onset of abdominal pain and lower-extremity eruption of diffuse urticarial plaques and palpable purpura

Predominantly affects patients 2–20 years of age, with 90% of patients being younger than 10 years old

A

Henoch-Schönlein Purpura (HSP)
(Small-Vessel Vasculitis)

Several environmental triggers precede HSP onset, such as viral (upper respiratory infections, hepatitis B virus, HCV, parvovirus B19, and HIV) and bacterial (Streptococcus sp., Staphylococcus aureus, and Salmonella) infection

38
Q

Features are predictive of renal involvement in HSP, requiring initiation of glucocorticoids

A
  • Persistent purpura
  • Severe abdominal symptoms, and
  • Diminished plasma coagulation factor XIII activity
39
Q

A hemorrhagic infarction syndrome consisting of disseminated intravascular coagulation (DIC), acral purpura, and shock, most commonly seen in immunocompromised hosts but can also occur in immunocompetent patients

Retiform purpuric lesions result from fibrin-induced microvascular occlusion and commonly have a rapid evolution toward necrosis and eschar formation

A

Purpura fulminans

It may be idiopathic or occur in infants with homozygous protein C or protein S defici

40
Q

TRUE OR FALSE

The finding of petechiae on a patient with symptoms and signs of bacterial meningitis is predictive of meningococcal meningitis

A

TRUE

The finding of petechiae on a patient with symptoms and signs of bacterial meningitis is predictive of meningococcal meningitis

41
Q

Borrelia burgdorferi infection causes a nonpruritic annular erythematous expanding plaque, occasionally including a central hemorrhagic bulla

A

Erythema migrans

Other reported cutaneous findings include papular urticaria, Henoch-Schönlein-like purpura, and morphea

42
Q

Disseminated strongyloidiasis is characterized by a serpiginous urticarial eruption, and other cutaneous lesions include generalized petechiae and widespread reticular purpura

A

Larva currens

43
Q

A cutaneous disorder characterized by the development of crops of well-demarcated, erythematous target lesions with central clearing, representing a hypersensitivity reaction triggered by infection or drug exposure

Caused by a cellular allergic reaction coupled with impaired histamine metabolism due to decreased histamine-N-methyltransferase activity

A

Erythema multiforme (EM)

44
Q

Erythema multiforme ranges in severity:

A
  • Mild (EM minor)
  • Severe (EM major or Stevens-Johnson syndrome)

Can be triggered by a number of viruses (most commonly herpes simplex) and medications

45
Q

Lesions develop as tender erythematous nodules, with occasional retiform purpura and livedo reticularis localized to the upper and lower extremities, but can also involve the trunk, neck, and face

Histologic analysis shows deep dermal artery necrosis with infiltration of neutrophils and eosinophils, and fibrin deposition

A

Cutaneous polyarteritis nodosa

46
Q

Most commonly associated with hematologic neoplasia and is usually a result of paraproteinemia, but can also be associated with carcinomas of the lung, colon, breast, and cervix

A

Paraneoplastic vasculitis

Cutaneous manifestations include petechiae, urticaria, and palpable purpura, often intensely pruritic

In hematologic disorders, these lesions often precede the development of malignancy by an average of 10 months

Histologic examination of paraneoplastic vasculitis shows necrotizing leukocytoclastic vasculitis with neutrophilic infiltration

47
Q

Drugs reported to cause vasculitis

A
  • Allopurinol
  • Cefaclor
  • Colony-stimulating factors
  • D-penicillamine
  • Furosemide
  • Hydralazine
  • Isotretinoin
  • Methotrexate
  • Phenytoin
  • Minocycline
  • Propylthiouracil
48
Q

Small- to medium-vessel vasculitis most commonly affecting the upper and lower respiratory tracts and kidneys, strongly associated with the development of circulating antineutrophil cytoplasmic antibodies

Histologic findings: Necrotizing vasculitis, palisading granulomas, and granulomatous vasculitis

A

Wegener Granulomatosis

49
Q

Characterized by granulomatous inflammation in the lungs associated with asthma and eosinophilia

Cutaneous findings: ulcers, papules, palpable purpura, cutaneous nodules, and infarcts of fingers and toes

A

Churg-Strauss Syndrome

50
Q

Characterized by cervicofacial cyanosis and swelling, petechiae, and subconjunctival hemorrhage

A

Traumatic asphyxia

51
Q

Easy bruising seen in aged and sun-damaged skin, commonly appearing on the dorsal aspect of the hands and forearms

A

Senile Purpura

Proposed etiology of Senile Purpura
Degeneration of skin extracellular matrix components that leaves dermal capillaries unsupported and vulnerable to shearing injuries, but zinc deficiency is also suspected

52
Q

Periorbital “pinch purpura,” “raccoon eyes,” macroglossia, and waxy, purpuric cutaneous and mucocutaneous lesion

A

Cutaneous manifestations of Systemic Amyloidosis

53
Q

Rare autosomal dominant syndrome resulting from a mutation in collagen synthesis, tenascin X, or lysyl hydroxylase, leading to loss of skin elasticity, delayed wound healing, easy bruising, joint hypermobility, and systemic organ and tissue fragility

Thin skin and a tendency to develop nonpalpable purpuric lesions

A

Ehlers-Danlos Syndrome

54
Q

Genetic disorder characterized by mineralization and fragmentation of elastin in the skin, retina, and blood vessels, associated with a mutation in the ABCC6 gene

Small white or yellow papules classically appearing on the neck in a “gooseflesh” aspect, but systemic hemorrhagic events are also encountered

A

Pseudoxanthoma Elasticum

55
Q

Associated with a mutation in a mitochondrial transfer RNA or the reduced form of nicotinamide adenine dinucleotide dehydrogenase complex I

Nonpalpable purpuric lesions on the palms and soles, as well as hypertrichosis, ichthyosis, and vitiligo

A

MELAS Syndrome

Mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes

56
Q

Autosomal dominant hereditary disorder characterized by local angiodysplasia, mostly present in the skin, mucous membranes, and often in organs such as the lungs, liver, and brain

Telangiectasias (small vascular malformations) in the skin, and subcutaneous bleeding presenting as nonpalpable purpuric lesions

A

Rendu-Osler-Weber Disease (Hereditary Hemorrhagic Telangiectasia)

57
Q

The female easy-bruising syndrome is purpura or ecchymoses occurring predominantly in women, frequently on the thighs.

This may be related to hormonal changes and can be aggravated by nonsteroidal anti-inflammatory drug (NSAID) ingestion.

A

Purpura simplex

58
Q

Diseases that are characterized by petechiae and purpura on a background of redbrown or orange hyperpigmentation, usually on the lower extremities

A

Schamberg and Majocchi diseases

59
Q

Purpura or ecchymoses may develop around the umbilicus (____________ sign) or in the flanks (_____________ sign) in patients with intra-abdominal hemorrhage

A

Cullen sign

Grey-Turner sign

60
Q

This disorder is composed of a triad of fever; iris-like or medallion-like large purpuric, painful cutaneous lesions; and edema appearing in children aged 4 months to 2 years.

A

Acute Hemorrhagic Edema of Infancy

WILLIAM'S HEMATOLOGY (109 decks)

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