Chapter 16 - Part 2 Flashcards

1
Q

What term describes when grafted cells from transplants attack the recipient of the transplant?

A

Graft Versus Host Disease

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2
Q

Why are systemic signs of GVHD varied?

A

It depends on the organ system involved and the severity of the disease

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3
Q

What things will help minimize the severity of GVHD?

A

A good histocompatibility match and patients who are young, female or patients that have received cord blood

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4
Q

What are the two types of GVHD?

A

Acute and Chronic

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5
Q

When does acute GVHD

occur and who does it affect?

A

It is observed within about a month, but it is any reaction that occurs withing 100 days of the procedure.
Affects 50% of bone marrow transplant patients

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6
Q

What are the signs of acute GVHD?

A

Skin lesion – mild rash to diffuse, severe sloughing that resembles TEN
Diarrhea, nausea, vomiting, abdominal pain, liver dysfuntion, persisting ulcerations

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7
Q

When does chronic GVHD occur and who does it affect?

A

May be a continuation of actue GVHD, or develop later than 100 days after procedure (can appear up to several years after the procedure)
Affects up to 70% of bone marrow transplant patients

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8
Q

What are the signs of chronic GVHD?

A

Mimics a variety of autoimmune conditions like SLE or Sjogren

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9
Q

What are the oral manifestations of GVHD?

A

Fine, reticular netword of white striae which is indistinguishable from lichen planus, atrophy, ulcerations, xerostomia

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10
Q

What is the treatment

and prognosis for GVHD?

A

Prevent occurrence! Increase immunosuppressive drugs. Topical steroids can be used for focal oral ulcerations.
70% survive mild GVHD
15% survive severe

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11
Q

What term describes an immunologically mediated condition with several clinicopathologic forms, and is the most common collagen vascular or CT disease in the US?

A

Lupus Erythematous

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12
Q

What are the clinicopathologic forms of lupus erythematous?

A

Systemic lupus erythematosus, Chronic cutaneous lupus erythematosus, Subacute cutaneous lupus erythematosus

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13
Q

Why is systemic lupus erythematosus difficult to diagnose in early stages?

A

It is nonspecific and has periods of remission

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14
Q

Who does systemic lupus erythematosus most commonly affect?

A

Females (10:1 ratio!), average age of 30

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15
Q

What are some common findings associated with systemic lupus erythematosus?

A

Fever, weight loss, arthritis, fatigue, malar rash

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16
Q

What is characteristics about the malar rash?

A

It is the pattern of a butterfly that develops on the malar area of the nose but not the nasolabial folds, BUT worsens in the sunlight

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17
Q

What other complications of the disease?

A

Kidney (and kidney failure), CV complications likfe pericarditis and Libman-Sacks endocarditis

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18
Q

How many systemic lupus erythematosus patients have oral lesions and what do they appear as?

A

About 40%, and the may appear as lichenoid lesions

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19
Q

What are the characteristics of chronic cutaneous lupus erythematosus?

A

Few or no systemic signs, just lesions limited to the skin or mucosal surfaces

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20
Q

Describe the pathogenesis of the skin lesions associated with chronic cutaneous lupus erythematosus?

A

Begin as scaly erythematous patches on sun exposed skin (discoid lupus erythematous) –> heal and recur –>healing leads to cutaneous atrophy, scarring and altered pigmentation

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21
Q

What do oral lesions associated with chronic cutaneous lupus erythematosus resemble?

A

Lichen planus

22
Q

How is lupus erythematous diagnosed?

A

Through clinical, microscopic features and immunological studies

23
Q

What are the immunological studies for lupus erythematous?

A

Positive lupus band test = DIF showing IgG, IgM or C3 at the basment membrane [not specific]
90% have anti-nuclear antibodies [not specific]
70% have Anti-dsDNA
30% have Anti-Sm (Sm = protein in small nuclear RNA) [very specific]

24
Q

How is systemic lupus erythematosus treated?

A

Avoid excessive sunlight
mild = NSAIDs and antimalarials (hydoxychlorquine)
Severe = systemic corticosteroids and immunosuppressive agents

25
Q

How is chronic cutaneous lupus erythematosus treated?

A

Avoid excessive sunlight, treat skin lesions with topical corticosteroids

26
Q

What is the prognosis of systemic lupus erythematosus?

A

5 year = 90%
20 year = 70%
Most common cause of death is renal failure
Prognosis better for women and caucasians than men and african americans

27
Q

What is the prognosis of chronic cutaneous lupus erythematosus?

A

50% of cases resolve spontaneously

5% progress to systemic lupus erytheamtosus

28
Q

What term describes a disease in which dense collagen is deposited in the tissues of the body in the extraordinary amounts?

A

Systemic sclerosis

29
Q

Who does systemic sclerosis commonly affect?

A

Females (5:1), aduls

30
Q

What is the first sign of systemic sclerosis?

A

Raynaud’s phenomenon - vasoconstrictive event triggered by emotional event or exposure to colde (*this is not specific for systemic sclerosis)

31
Q

What are some other signs of systemic sclerosis?

A

Resorption of the terminal phlanges, flexion contractures –> clawlike fingers, vascular events –> fingertip ulcerations, hard skin with a smooth surface, atrophy of the nasal alae, xerostomia, microstomia

32
Q

What is the most serious concern of systemic sclerosis?

A

Involvement of organs! Fibrosis of lung, heart, kindey, GI tract –> failure within 3 years

33
Q

What is the primary cause of death for patients with systemic sclerosis?

A

Pulmonary fibrosis leading to pulmonary HTN and heart failure

34
Q

What is microstomia and how is it related to systemic sclerosis?

A

A 70% reduced opening of the mouth due to collagen deposition in the perioral tissues – characteristic furrows radiating from the mouth produce a purse string appearance

35
Q

What term describes a mild variant of systemic sclerosis and what is it?

A

Localized scleroderma, and it affects a solitary patch of skin –> looks like scar (en coup de sabre)

36
Q

How is systemic sclerosis managed?

A

Hard to manage b/c condition wasex and wanes

Dental appliances for microstomia

Poor prognosis - 10 year survival = 60%

37
Q

What is CREST syndrome?

A

Calcinosis cutis, Raynaud’s phenomenon, Esophageal dysfunction, Sclerodactyly, Telangiaectasia

38
Q

Who does CREST syndrome most commonly affect?

A

Women in their 60s

39
Q

What is calcinosis cutis?

A

Multiple, movable, nontender, subcutaneous, nodular calcifications

40
Q

What is raynaud’s phenomenon? (CREST)

A

Vasoconstriction when hands or feet are exposed to cold –> dramatic blanching then turn blue
After warming digits turn red and have varying degrees of throbbing pain

41
Q

Why does esophageal dysfunction occur with CREST syndrome?

A

Caused by abnormal collagen deposition in the esophageal submucosa

42
Q

What is sclerodactyly? (CREST)

A

Fingers become stiff, skin becomes smooth and shiny –> permanent flexure due to abnormal deposition of collagen within the dermis

43
Q

What is telangiectasias? (CREST)

A

Numerous scattered red papules 1-2 mm in size that will blacnh with pressure (b/c they are dilated capillaries = bleeding)
Most frequently found on the vermillion zone of lips and facial skin

44
Q

What is the treatment of CREST syndrome?

A

Same as systemic sclerosis, and patients should be monitored for pulmonary hypertension (usually will occur 10 years after dx)

45
Q

What term describes an acquired dermatologic problem with velvety, brownish alterations of the skin?

A

Acanthosis Nigricans

46
Q

What can acanthosis nigricans be seen in conjunction with?

A

Gastrointestinal cancer in 20%, acanthosis associated with these are termed malignant acanthosis

47
Q

What may cause acanthosis nigricans?

A

Obesity (psuedoacanthosis nigricans), inheritance, various endocrinopathies (DM), various syndromes (Crouzon), various drugs (contraceptives)

48
Q

Where does both benign and malignant acanthosis nigricans occur?

A

The flexural areas of the skin

49
Q

Can oral lesions occur with acanthosis nigricans?

A

Yes, in up to 50% and especially in those with the malignant form

50
Q

What do oral lesions of acanthosis nigricans appear as?

A

Diffuse, finely papillary areas of mucosal alteration that most often involve the tongue or lips (esp. upper lip) but usually not brown

51
Q

What is the treatment of acanthosis nigricans?

A

The condition itself is harmless but patients should be evaluated for the presence of disease or malignancy. Those with malignant acanthosis have a poor prognosis