Chapter 16: Dermatologic Diseases III Flashcards
graft-versus-host disease occurs in recipients of ___
allogeneneic bone marrow transplantations
how does graft-versus-host disease occur?
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
systemic signs of graft-versus-host disease are varied, depending on ___ and ___
the organ system involved and the severity of disease
mild graft-versus-host disease is more common in what patients?
- have better histocompatibility match
- are young
- female
- have received cord blood
is graft-versus-host disease acute or chronic?
can be either
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
acute
acute graft-versus-host disease affects ___% of bone marrow transplant patients?
50%
describe the skin lesions in acute graft-versus-host disease
range from a mild rash to diffuse and severe sloughing that resembles toxic epidermal necrolysis
in addition to skin lesions, what other issues might acute graft-versus-host disease patients have?
- diarrhea
- nausea
- vomiting
- abdominal pain
- liver dysfunction
describe ulcerations associated with acute graft-versus-host disease
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
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?
chronic
chronic graft-versus-host disease may represent a continuation of ___ or it may develop ___
- acute graft-versus-host disease
- may develop later than 100 days after bone marrow transplant
chronic graft-versus-host disease develops in up to ___% of bone marrow transplant patients
70%
___% of patients with acute graft-versus-host disease have oral involvement
50%
___% of patients with chronic graft-versus-host disease have oral involvement
80%
describe the oral involvement of patients with chronic graft-versus-host disease
- 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
what is the treatment for graft-versus-host disease?
- 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
what is the prognosis of patients with graft-versus-host disease?
depends on the extent to which the condition progresses and whether it can be controlled
more than ___% of pateints survive mild graft-versus-host disease
___% survive severe graft-versus-host disease
- 70%
- 15%
graft-versus-host disease
___ is a classic example of an immunologically mediated condition, and is the most common collagen vascular or connective tissue disease in the US
lupus erythematous
What are the 4 clinicopathologic forms of lupus erythematous?
- systemic lupus erythematous
- chronic cutaneous lupus erythematous
- subacute cutaneous lupus erythematous
- intermediate features between systemic and cutaneous lupus erythematous
which type of lupus erythematous is difficult to diagnose in early stages because it is nonspecific and has periods of remission?
systemic lupus erythematous
what is the gender predilection and average age for systemic lupus erythematous?
- 10x more common in females
- average age = 30
what are the 5 common findings in systemic lupus erythematous?
- fever
- weight loss
- arthritis
- fatigue
- malar rash
what percent of patients with systemic lupus erythematous have a malar rash?
50%
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
___ makes malar rash lesions worse (systemic lupus erythematous)
sunlight
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
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
oral lesions are seen in up to ___% of systemic lupus erythematous patients; describe these lesions
- 40%
- nonspecific
- may appear as lichenoid lesions
systemic lupus erythematous
which type of lupus erythematous has few or no systemic signs?
chronic cutaneous lupus erythematous
chronic cutaneous lupus erythematous lesions are limited to ___
skin or mucosal surfaces
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
chronic cutaneous lupus erythematous lesions may heal and recur; healing leads to ___
cutaneous atrophy, scarring, and altered pigmentation
chronic cutaneous lupus erythematous oral lesions, if present with the skin lesions, resemble ___
lichen planus
chronic cutaneous lupus erythematous
in addition to clinical and microscopic features, ___ are helpful in making the diagnosis of lupus erythematous
immunologic studies
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
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
what is the treatment for chronic cutaneous lupus erythematous?
- patients should avoid excessive sunlight
- treated effectively with topical corticosteroids
what is the 5-year and 20-year survival prognosis for systemic lupus erythematous?
- 5-year = 90%
- 20-year = 70%
what is the most common cause of death for patients with systemic lupus erythematous?
kidney failure
the prognosis for patients with systemic lupus erythematous is better for ___ compared to ___
- women and caucasians
- men and african americans
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
___ is a disease in which dense collagen is deposited in the tissues of the body in extraordinary amounts
systemic sclerosis
what is the gender predilection and common age of patients with systemic sclerosis?
- 5x more common in females
- disease of adults
the first sign of systemic sclerosis is ___
raynaud’s phenomenon
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
patients with ___ experience resorption of the terminal phalanges and flexion contracture to produce shortened, clawlike fingers
systemic sclerosis
___ and ___ contribute to fingertip ulcerations in systemic sclerosis
vascular events and abnormal collagen deposition
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
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
describe pulmonary fibrosis associated with systemic sclerosis
- leads to pulmonary hypertension and heart failure
- this is the primary cause of death for these patients
in patients with systemic sclerosis, ___ occurs as a result of collagen deposition in the perioral tissues
microstomia (“small mouth”)
describe microstomia associated with systemic sclerosis
- patients have a 70% reduced opening
- characteristic furrows radiating from the mouth produce a “purse string” appearance
___ is frequently encountered with patients with systemic sclerosis
xerostomia
the mild variant of systemic sclerosis is called ___
localized scleroderma
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
describe the management of systemic sclerosis
- management is difficult
- corticosteroids are of little benefit
- dental appliances are used to combat microstomia
why is the effective of treatment of systemic sclerosis difficult to assess?
the condition waxes and wanes
what is the prognosis for patients with systemic sclerosis?
poor unless patients only have limited cutaneous scleroderma
what is the 10 year survival rate for patients with systemic sclerosis? what about limited cutaneous scleroderma?
- sysetmic sclerosis = 60%
- limited cutaneous scleroderma = 90%
systemic sclerosis
clawlike fingers and fingertip ulcerations
systemic sclerosis
smooth, taut, masklike facies
atrophied nasal alae
microstomia
localized scleroderma (mild variant of systemic sclerosis)
en coup de sabre
systemic sclerosis
widening of the PDL, resorption of ramus, condyle, and/or coronoid process
CREST syndrome is a system complex characterized by what 5 things?
- Calcinosis cutis
- Raynaud’s phenomenon
- Esophageal dysfunction
- Sclerodactyly
- Telangiectasia
CREST syndrome
calcinosis cutis
CREST syndrome
sclerodactyly
CREST syndrome
telangiectastic blood vessels
CREST syndrome
most patients affected by CREST syndrome are what age?
in their 60s
describe calcinosis cutis associated with CREST syndrome
multiple, movable, nontender, subcutaneous, nodular calcifications
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
describe the esophageal dysfunction associated with CREST syndrome
caused by abnormal collagen deposition in the esophageal submucosa
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)
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
what is the treatment for CREST syndrome?
same as for systemic sclerosis, but CREST syndrome is not as severe
patients with CREST syndrome should be monitored for ___
pulmonary hypertension
if it occurs, it is usually 10+ years after initial diagnosis
___ is an acquired dermatologic problem characterized by the development of a velvety, brownish alteration of the skin
acanthosis nigricans
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
are most cases of acanthosis nigricans benign or malignant?
benign
benign acanthosis nigricans is seen in ___% of adults
5%
what 5 things can cause benign acanthosis nigricans?
- obesity
- inheritance
- various endocrinopathies (like diabetes mellitus)
- various syndromes (like crouzon)
- various drugs (like oral contraceptives)
benign acanthosis nigricans that is caused by obesity is termed ___
pseudoacanthosis nigricans
both benign and malignant acanthosis nigricans affect the ___ areas of the skin
flexural
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
oral lesions of acanthosis nigricans may occur in up to ___%; they especially affect those with which form (malignant/benign)?
- 50%
- malignant
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
T or F:
the acanthosis nigricans condition is harmless
true
patients with acanthosis nigricans should be evaluated for the presense of ___ or ___
disease or malignancy
what is the prognosis for patients with malignant acanthosis nigricans?
poor
malignant acanthosis nigricans lesions resolve with ___ treatment
cancer
acanthosis nigricans
acanthosis nigricans
