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Oral and Maxillofacial Pathology > Chapter 16: Dermatologic Diseases III > Flashcards

Chapter 16: Dermatologic Diseases III Flashcards

1
Q

graft-versus-host disease occurs in recipients of ___

A

allogeneneic bone marrow transplantations

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2
Q

how does graft-versus-host disease occur?

A

when patients receive allogeneic bone marrow transplantations and the match isn’t exact, the engrafted cells recognize that they aren’t in their own environment and attack what they perceive as a foreign body

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3
Q

systemic signs of graft-versus-host disease are varied, depending on ___ and ___

A

the organ system involved and the severity of disease

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4
Q

mild graft-versus-host disease is more common in what patients?

A
  • have better histocompatibility match
  • are young
  • female
  • have received cord blood
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5
Q

is graft-versus-host disease acute or chronic?

A

can be either

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6
Q

which type of graft-versus-host disease is characterized by the following:

  • observed within about a month
    • arbitrarily defined as occurring within 100 days after the procedure
A

acute

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7
Q

acute graft-versus-host disease affects ___% of bone marrow transplant patients?

A

50%

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8
Q

describe the skin lesions in acute graft-versus-host disease

A

range from a mild rash to diffuse and severe sloughing that resembles toxic epidermal necrolysis

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9
Q

in addition to skin lesions, what other issues might acute graft-versus-host disease patients have?

A
  • diarrhea
  • nausea
  • vomiting
  • abdominal pain
  • liver dysfunction
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10
Q

describe ulcerations associated with acute graft-versus-host disease

A

ulcerations may develop as a result of the chemotherapy and neutropenic state during the first 2 weeks after bone marrow transplant; those that persist represent acute graft-versus-host disease

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11
Q

which type of graft-versus-host disease can appear up to several years after the procedure and mimics a variety of autoimmune conditions, such as systemic lupus erythematous or sjogren syndrome?

A

chronic

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12
Q

chronic graft-versus-host disease may represent a continuation of ___ or it may develop ___

A
  • acute graft-versus-host disease
  • may develop later than 100 days after bone marrow transplant
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13
Q

chronic graft-versus-host disease develops in up to ___% of bone marrow transplant patients

A

70%

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14
Q

___% of patients with acute graft-versus-host disease have oral involvement

A

50%

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15
Q

___% of patients with chronic graft-versus-host disease have oral involvement

A

80%

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16
Q

describe the oral involvement of patients with chronic graft-versus-host disease

A
  • most patients have a fine, reticular network of white striae which is indistinguishable from lichen planus
  • atrophy or ulceration may also occur
  • xerostomia is a common complaint
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17
Q

what is the treatment for graft-versus-host disease?

A
  • primary strategy is to prevent occurrence
  • if graft-versus-host disease develops, immunosuppressive drug doses are increased
  • topical corticosteroids may be used for focal oral ulcerations
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18
Q

what is the prognosis of patients with graft-versus-host disease?

A

depends on the extent to which the condition progresses and whether it can be controlled

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19
Q

more than ___% of pateints survive mild graft-versus-host disease

___% survive severe graft-versus-host disease

A
  • 70%
  • 15%
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20
Q
A

graft-versus-host disease

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21
Q

___ is a classic example of an immunologically mediated condition, and is the most common collagen vascular or connective tissue disease in the US

A

lupus erythematous

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22
Q

What are the 4 clinicopathologic forms of lupus erythematous?

A
  • systemic lupus erythematous
  • chronic cutaneous lupus erythematous
  • subacute cutaneous lupus erythematous
  • intermediate features between systemic and cutaneous lupus erythematous
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23
Q

which type of lupus erythematous is difficult to diagnose in early stages because it is nonspecific and has periods of remission?

A

systemic lupus erythematous

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24
Q

what is the gender predilection and average age for systemic lupus erythematous?

A
  • 10x more common in females
  • average age = 30
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25
Q

what are the 5 common findings in systemic lupus erythematous?

A
  • fever
  • weight loss
  • arthritis
  • fatigue
  • malar rash
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26
Q

what percent of patients with systemic lupus erythematous have a malar rash?

A

50%

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27
Q

describe the malar rash that is characteristic of systemic lupus erythematous

A

characteristic rash with the pattern of a butterfly that develops on the malar area and nose but spares the noasolabial folds

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28
Q

___ makes malar rash lesions worse (systemic lupus erythematous)

A

sunlight

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29
Q

kidneys are affected in up to ___% of the systemic lupus erythematous patients; what can this complication lead to?

A
  • 50%
  • may lead to kidney failure and is the most significant aspect of the disease
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30
Q

what are the cardiac complications associated with systemic lupus erythematous?

A
  • most common = pericarditis
  • up to 50% have warty vegetations affecting heart valves termed libman-sacks endocarditis
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31
Q

oral lesions are seen in up to ___% of systemic lupus erythematous patients; describe these lesions

A
  • 40%
  • nonspecific
  • may appear as lichenoid lesions
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32
Q
A

systemic lupus erythematous

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33
Q

which type of lupus erythematous has few or no systemic signs?

A

chronic cutaneous lupus erythematous

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34
Q

chronic cutaneous lupus erythematous lesions are limited to ___

A

skin or mucosal surfaces

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35
Q

describe the skin lesions associated with chronic cutaneous lupus erythematous

A
  • present as discoid lupus erythematous
  • begin as scaly, erythematous patches on sun-exposed skin
  • especially affect the head and neck
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36
Q

chronic cutaneous lupus erythematous lesions may heal and recur; healing leads to ___

A

cutaneous atrophy, scarring, and altered pigmentation

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37
Q

chronic cutaneous lupus erythematous oral lesions, if present with the skin lesions, resemble ___

A

lichen planus

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38
Q
A

chronic cutaneous lupus erythematous

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39
Q

in addition to clinical and microscopic features, ___ are helpful in making the diagnosis of lupus erythematous

A

immunologic studies

40
Q

what are the immunologic studies that are helpful in diagnosing lupus erythematous?

A
  • direct immunofluorescense of clinically normal skin will show IgG, IgM, or C3 at the basement membrane
    • termed “positive lupus band test”
    • not specific - other conditions may have this too
  • 90% have anti-nuclear antibodies (ANAs) - not specific, but very sensitive
  • anti-dsDNA (70%)
  • anti-Sm (30% - VERY specific)
    • Sm is a protein complexed with small nuclear RNA
41
Q

what is the treatment of systemic lupus erythematous?

A
  • patients should avoid excessive sunlight
  • mild disease is managed by NSAIDs and antimalarial drugs, like hydroxychloroquine
  • more severe cases are treated with systemic corticosteroids and other immunosuppressive agents
42
Q

what is the treatment for chronic cutaneous lupus erythematous?

A
  • patients should avoid excessive sunlight
  • treated effectively with topical corticosteroids
43
Q

what is the 5-year and 20-year survival prognosis for systemic lupus erythematous?

A
  • 5-year = 90%
  • 20-year = 70%
44
Q

what is the most common cause of death for patients with systemic lupus erythematous?

A

kidney failure

45
Q

the prognosis for patients with systemic lupus erythematous is better for ___ compared to ___

A
  • women and caucasians
  • men and african americans
46
Q

what is the prognosis for chronic cutaneous lupus erythematous?

A
  • much better prognosis than systemic lupus erythematous, but can be a nuisance
  • 50% of cases resolve spontaneously
  • 5% progress to systemic lupus erythematous
47
Q

___ is a disease in which dense collagen is deposited in the tissues of the body in extraordinary amounts

A

systemic sclerosis

48
Q

what is the gender predilection and common age of patients with systemic sclerosis?

A
  • 5x more common in females
  • disease of adults
49
Q

the first sign of systemic sclerosis is ___

A

raynaud’s phenomenon

50
Q

what is raynaud’s phenomenon?

A
  • 1st sign of systemic sclerosis
  • vasoconstrictive event triggered by emotional distress or exposure to cold
  • not specific for systemic sclerosis
51
Q

patients with ___ experience resorption of the terminal phalanges and flexion contracture to produce shortened, clawlike fingers

A

systemic sclerosis

52
Q

___ and ___ contribute to fingertip ulcerations in systemic sclerosis

A

vascular events and abnormal collagen deposition

53
Q

describe the skin changes in systemic sclerosis

A
  • skin develops a diffuse, hard texture with a smooth surface
  • involvement of the facial skin results in a characteristic facial appearance - subcutaneous collagen deposition results in smooth, taut, masklike facies
  • nasal alae become atrophied, resulting in a pinched appearance
54
Q

describe involvement of organs in systemic sclerosis

A

involvement of organs are serious (leads to failure within 3 years) and can lead to fibrosis of lung, heart, kidney, and GI tract

55
Q

describe pulmonary fibrosis associated with systemic sclerosis

A
  • leads to pulmonary hypertension and heart failure
  • this is the primary cause of death for these patients
56
Q

in patients with systemic sclerosis, ___ occurs as a result of collagen deposition in the perioral tissues

A

microstomia (“small mouth”)

57
Q

describe microstomia associated with systemic sclerosis

A
  • patients have a 70% reduced opening
  • characteristic furrows radiating from the mouth produce a “purse string” appearance
58
Q

___ is frequently encountered with patients with systemic sclerosis

A

xerostomia

59
Q

the mild variant of systemic sclerosis is called ___

A

localized scleroderma

60
Q

describe localized scleroderma

A
  • mild variant of systemic sclerosis
  • affects solitary patch of skin
  • lesions look like scars, so they are described as en coup de sabre (“strike of the sword”)
  • condition is typically purely cosmetic
61
Q

describe the management of systemic sclerosis

A
  • management is difficult
  • corticosteroids are of little benefit
  • dental appliances are used to combat microstomia
62
Q

why is the effective of treatment of systemic sclerosis difficult to assess?

A

the condition waxes and wanes

63
Q

what is the prognosis for patients with systemic sclerosis?

A

poor unless patients only have limited cutaneous scleroderma

64
Q

what is the 10 year survival rate for patients with systemic sclerosis? what about limited cutaneous scleroderma?

A
  • sysetmic sclerosis = 60%
  • limited cutaneous scleroderma = 90%
65
Q
A

systemic sclerosis

clawlike fingers and fingertip ulcerations

66
Q
A

systemic sclerosis

smooth, taut, masklike facies

atrophied nasal alae

microstomia

67
Q
A

localized scleroderma (mild variant of systemic sclerosis)

en coup de sabre

68
Q
A

systemic sclerosis

widening of the PDL, resorption of ramus, condyle, and/or coronoid process

69
Q

CREST syndrome is a system complex characterized by what 5 things?

A
  • Calcinosis cutis
  • Raynaud’s phenomenon
  • Esophageal dysfunction
  • Sclerodactyly
  • Telangiectasia
70
Q
A

CREST syndrome

calcinosis cutis

71
Q
A

CREST syndrome

sclerodactyly

72
Q
A

CREST syndrome

telangiectastic blood vessels

73
Q
A

CREST syndrome

74
Q

most patients affected by CREST syndrome are what age?

A

in their 60s

75
Q

describe calcinosis cutis associated with CREST syndrome

A

multiple, movable, nontender, subcutaneous, nodular calcifications

76
Q

describe raynaud’s phenomenon associated with CREST syndrome

A
  • observed when hands or feet are exposed to cold temperatures
  • initial sign is a dramatic blanching; digits appear white as a result of severe vasospasm
  • a few minutes later, the affected areas turn blue due to venous stasis
  • after warming, the digits turn red with the return of hyperemic blood flow; accompanied by varying degrees of throbbing pain
77
Q

describe the esophageal dysfunction associated with CREST syndrome

A

caused by abnormal collagen deposition in the esophageal submucosa

78
Q

describe the sclerodactyly associated with CREST syndrome

A
  • fingers become stiff
  • skin becomes smooth and shiny
  • fingers undergo permanent flexure, resulting in a characteristic “claw” deformity
  • the change is due to abnormal deposition of collagen within the dermis (like sysetmic sclerosis)
79
Q

describe telangiectasias associated with CREST syndrome

A
  • numerous scattered red papules, 1-2mm in size
  • blanch (indicates the red color is due to blood contained within blood vessels)
  • in this case, blood is contained in small collections of dilated capillaries termed telangiectasias that are close to the surface of the mucosa
  • most frequently found on the vermillion zone of lips and facial skin
  • significant bleeding may occur
80
Q

what is the treatment for CREST syndrome?

A

same as for systemic sclerosis, but CREST syndrome is not as severe

81
Q

patients with CREST syndrome should be monitored for ___

A

pulmonary hypertension

if it occurs, it is usually 10+ years after initial diagnosis

82
Q

___ is an acquired dermatologic problem characterized by the development of a velvety, brownish alteration of the skin

A

acanthosis nigricans

83
Q

describe acanthosis nigricans that is seen in conjunction with gastrointestinal cancer

A
  • termed malignant acanthosis
  • the cutaneous lesion is benign
  • leads to discovery of malignancy in 20% of cases
84
Q

are most cases of acanthosis nigricans benign or malignant?

A

benign

85
Q

benign acanthosis nigricans is seen in ___% of adults

A

5%

86
Q

what 5 things can cause benign acanthosis nigricans?

A
  • obesity
  • inheritance
  • various endocrinopathies (like diabetes mellitus)
  • various syndromes (like crouzon)
  • various drugs (like oral contraceptives)
87
Q

benign acanthosis nigricans that is caused by obesity is termed ___

A

pseudoacanthosis nigricans

88
Q

both benign and malignant acanthosis nigricans affect the ___ areas of the skin

A

flexural

89
Q

describe the flexural areas of the skin that are affected by acanthosis nigricans

A
  • appear as finely papillary, hyperkeratotic, brown patches which are asymptomatic
  • the texture is either velvety or leathery
90
Q

oral lesions of acanthosis nigricans may occur in up to ___%; they especially affect those with which form (malignant/benign)?

A
  • 50%
  • malignant
91
Q

describe oral lesions associated with acanthosis nigricans

A
  • lesions appear as diffuse, finely papillary areas of mucosal alteration that most often involve the tongue or lips (especially the upper lip)
  • the brownish pigmentation associated with the cutaneous lesions is usually not seen in the oral lesions
92
Q

T or F:

the acanthosis nigricans condition is harmless

A

true

93
Q

patients with acanthosis nigricans should be evaluated for the presense of ___ or ___

A

disease or malignancy

94
Q

what is the prognosis for patients with malignant acanthosis nigricans?

A

poor

95
Q

malignant acanthosis nigricans lesions resolve with ___ treatment

A

cancer

96
Q
A

acanthosis nigricans

97
Q
A

acanthosis nigricans

Oral and Maxillofacial Pathology (45 decks)

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