Chapter 16: Dermatologic Diseases III Flashcards
(97 cards)
1
Q
graft-versus-host disease occurs in recipients of ___
A
allogeneneic bone marrow transplantations
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
3
Q
systemic signs of graft-versus-host disease are varied, depending on ___ and ___
A
the organ system involved and the severity of disease
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
5
Q
is graft-versus-host disease acute or chronic?
A
can be either
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
7
Q
acute graft-versus-host disease affects ___% of bone marrow transplant patients?
A
50%
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
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
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
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
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
13
Q
chronic graft-versus-host disease develops in up to ___% of bone marrow transplant patients
A
70%
14
Q
___% of patients with acute graft-versus-host disease have oral involvement
A
50%
15
Q
___% of patients with chronic graft-versus-host disease have oral involvement
A
80%
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
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
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
19
Q
more than ___% of pateints survive mild graft-versus-host disease
___% survive severe graft-versus-host disease
A
- 70%
- 15%
20
Q
A
graft-versus-host disease
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
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
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
24
Q
what is the gender predilection and average age for systemic lupus erythematous?
A
- 10x more common in females
- average age = 30
25
what are the 5 common findings in systemic lupus erythematous?
* fever
* weight loss
* arthritis
* fatigue
* malar rash
26
what percent of patients with systemic lupus erythematous have a malar rash?
50%
27
describe the malar rash that is characteristic of systemic lupus erythematous
characteristic rash with the pattern of a butterfly that develops on the malar area and nose but spares the noasolabial folds
28
\_\_\_ makes malar rash lesions worse (systemic lupus erythematous)
sunlight
29
kidneys are affected in up to \_\_\_% of the systemic lupus erythematous patients; what can this complication lead to?
* 50%
* may lead to kidney failure and is the most significant aspect of the disease
30
what are the cardiac complications associated with systemic lupus erythematous?
* most common = pericarditis
* up to 50% have warty vegetations affecting heart valves termed libman-sacks endocarditis
31
oral lesions are seen in up to \_\_\_% of systemic lupus erythematous patients; describe these lesions
* 40%
* nonspecific
* may appear as lichenoid lesions
32
systemic lupus erythematous
33
which type of lupus erythematous has few or no systemic signs?
chronic cutaneous lupus erythematous
34
chronic cutaneous lupus erythematous lesions are limited to \_\_\_
skin or mucosal surfaces
35
describe the skin lesions associated with chronic cutaneous lupus erythematous
* present as discoid lupus erythematous
* begin as scaly, erythematous patches on sun-exposed skin
* especially affect the head and neck
36
chronic cutaneous lupus erythematous lesions may heal and recur; healing leads to \_\_\_
cutaneous atrophy, scarring, and altered pigmentation
37
chronic cutaneous lupus erythematous oral lesions, if present with the skin lesions, resemble \_\_\_
lichen planus
38
chronic cutaneous lupus erythematous
39
in addition to clinical and microscopic features, ___ are helpful in making the diagnosis of lupus erythematous
immunologic studies
40
what are the immunologic studies that are helpful in diagnosing lupus erythematous?
* 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
what is the treatment of systemic lupus erythematous?
* 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
what is the treatment for chronic cutaneous lupus erythematous?
* patients should avoid excessive sunlight
* treated effectively with topical corticosteroids
43
what is the 5-year and 20-year survival prognosis for systemic lupus erythematous?
* 5-year = 90%
* 20-year = 70%
44
what is the most common cause of death for patients with systemic lupus erythematous?
kidney failure
45
the prognosis for patients with systemic lupus erythematous is better for ___ compared to \_\_\_
* women and caucasians
* men and african americans
46
what is the prognosis for chronic cutaneous lupus erythematous?
* much better prognosis than systemic lupus erythematous, but can be a nuisance
* 50% of cases resolve spontaneously
* 5% progress to systemic lupus erythematous
47
\_\_\_ is a disease in which dense collagen is deposited in the tissues of the body in extraordinary amounts
systemic sclerosis
48
what is the gender predilection and common age of patients with systemic sclerosis?
* 5x more common in females
* disease of adults
49
the first sign of systemic sclerosis is \_\_\_
raynaud's phenomenon
50
what is raynaud's phenomenon?
* 1st sign of systemic sclerosis
* vasoconstrictive event triggered by emotional distress or exposure to cold
* not specific for systemic sclerosis
51
patients with ___ experience resorption of the terminal phalanges and flexion contracture to produce shortened, clawlike fingers
systemic sclerosis
52
\_\_\_ and ___ contribute to fingertip ulcerations in systemic sclerosis
vascular events and abnormal collagen deposition
53
describe the skin changes in systemic sclerosis
* 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
describe involvement of organs in systemic sclerosis
involvement of organs are serious (leads to failure within 3 years) and can lead to fibrosis of lung, heart, kidney, and GI tract
55
describe pulmonary fibrosis associated with systemic sclerosis
* leads to pulmonary hypertension and heart failure
* this is the primary cause of death for these patients
56
in patients with systemic sclerosis, ___ occurs as a result of collagen deposition in the perioral tissues
microstomia ("small mouth")
57
describe microstomia associated with systemic sclerosis
* patients have a 70% reduced opening
* characteristic furrows radiating from the mouth produce a "purse string" appearance
58
\_\_\_ is frequently encountered with patients with systemic sclerosis
xerostomia
59
the mild variant of systemic sclerosis is called \_\_\_
localized scleroderma
60
describe localized scleroderma
* 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
describe the management of systemic sclerosis
* management is difficult
* corticosteroids are of little benefit
* dental appliances are used to combat microstomia
62
why is the effective of treatment of systemic sclerosis difficult to assess?
the condition waxes and wanes
63
what is the prognosis for patients with systemic sclerosis?
poor unless patients only have limited cutaneous scleroderma
64
what is the 10 year survival rate for patients with systemic sclerosis? what about limited cutaneous scleroderma?
* sysetmic sclerosis = 60%
* limited cutaneous scleroderma = 90%
65
systemic sclerosis
clawlike fingers and fingertip ulcerations
66
systemic sclerosis
smooth, taut, masklike facies
atrophied nasal alae
microstomia
67
localized scleroderma (mild variant of systemic sclerosis)
en coup de sabre
68
systemic sclerosis
widening of the PDL, resorption of ramus, condyle, and/or coronoid process
69
CREST syndrome is a system complex characterized by what 5 things?
* **C**alcinosis cutis
* **R**aynaud's phenomenon
* **E**sophageal dysfunction
* **S**clerodactyly
* **T**elangiectasia
70
CREST syndrome
calcinosis cutis
71
CREST syndrome
sclerodactyly
72
CREST syndrome
telangiectastic blood vessels
73
CREST syndrome
74
most patients affected by CREST syndrome are what age?
in their 60s
75
describe calcinosis cutis associated with CREST syndrome
multiple, movable, nontender, subcutaneous, nodular calcifications
76
describe raynaud's phenomenon associated with CREST syndrome
* 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
describe the esophageal dysfunction associated with CREST syndrome
caused by abnormal collagen deposition in the esophageal submucosa
78
describe the sclerodactyly associated with CREST syndrome
* 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
describe telangiectasias associated with CREST syndrome
* 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
what is the treatment for CREST syndrome?
same as for systemic sclerosis, but CREST syndrome is not as severe
81
patients with CREST syndrome should be monitored for \_\_\_
pulmonary hypertension
if it occurs, it is usually 10+ years after initial diagnosis
82
\_\_\_ is an acquired dermatologic problem characterized by the development of a velvety, brownish alteration of the skin
acanthosis nigricans
83
describe acanthosis nigricans that is seen in conjunction with gastrointestinal cancer
* termed malignant acanthosis
* the cutaneous lesion is benign
* leads to discovery of malignancy in 20% of cases
84
are most cases of acanthosis nigricans benign or malignant?
benign
85
benign acanthosis nigricans is seen in \_\_\_% of adults
5%
86
what 5 things can cause benign acanthosis nigricans?
* obesity
* inheritance
* various endocrinopathies (like diabetes mellitus)
* various syndromes (like crouzon)
* various drugs (like oral contraceptives)
87
benign acanthosis nigricans that is caused by obesity is termed \_\_\_
pseudoacanthosis nigricans
88
both benign and malignant acanthosis nigricans affect the ___ areas of the skin
flexural
89
describe the flexural areas of the skin that are affected by acanthosis nigricans
* appear as finely papillary, hyperkeratotic, brown patches which are asymptomatic
* the texture is either velvety or leathery
90
oral lesions of acanthosis nigricans may occur in up to \_\_\_%; they especially affect those with which form (malignant/benign)?
* 50%
* malignant
91
describe oral lesions associated with acanthosis nigricans
* 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
T or F:
the acanthosis nigricans condition is harmless
true
93
patients with acanthosis nigricans should be evaluated for the presense of ___ or \_\_\_
disease or malignancy
94
what is the prognosis for patients with malignant acanthosis nigricans?
poor
95
malignant acanthosis nigricans lesions resolve with ___ treatment
cancer
96
acanthosis nigricans
97
acanthosis nigricans
