Chapter 16 - Part 2 Flashcards
What term describes when grafted cells from transplants attack the recipient of the transplant?
Graft Versus Host Disease
Why are systemic signs of GVHD varied?
It depends on the organ system involved and the severity of the disease
What things will help minimize the severity of GVHD?
A good histocompatibility match and patients who are young, female or patients that have received cord blood
What are the two types of GVHD?
Acute and Chronic
When does acute GVHD
occur and who does it affect?
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
What are the signs of acute GVHD?
Skin lesion – mild rash to diffuse, severe sloughing that resembles TEN
Diarrhea, nausea, vomiting, abdominal pain, liver dysfuntion, persisting ulcerations
When does chronic GVHD occur and who does it affect?
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
What are the signs of chronic GVHD?
Mimics a variety of autoimmune conditions like SLE or Sjogren
What are the oral manifestations of GVHD?
Fine, reticular netword of white striae which is indistinguishable from lichen planus, atrophy, ulcerations, xerostomia
What is the treatment
and prognosis for GVHD?
Prevent occurrence! Increase immunosuppressive drugs. Topical steroids can be used for focal oral ulcerations.
70% survive mild GVHD
15% survive severe
What term describes an immunologically mediated condition with several clinicopathologic forms, and is the most common collagen vascular or CT disease in the US?
Lupus Erythematous
What are the clinicopathologic forms of lupus erythematous?
Systemic lupus erythematosus, Chronic cutaneous lupus erythematosus, Subacute cutaneous lupus erythematosus
Why is systemic lupus erythematosus difficult to diagnose in early stages?
It is nonspecific and has periods of remission
Who does systemic lupus erythematosus most commonly affect?
Females (10:1 ratio!), average age of 30
What are some common findings associated with systemic lupus erythematosus?
Fever, weight loss, arthritis, fatigue, malar rash
What is characteristics about the malar rash?
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
What other complications of the disease?
Kidney (and kidney failure), CV complications likfe pericarditis and Libman-Sacks endocarditis
How many systemic lupus erythematosus patients have oral lesions and what do they appear as?
About 40%, and the may appear as lichenoid lesions
What are the characteristics of chronic cutaneous lupus erythematosus?
Few or no systemic signs, just lesions limited to the skin or mucosal surfaces
Describe the pathogenesis of the skin lesions associated with chronic cutaneous lupus erythematosus?
Begin as scaly erythematous patches on sun exposed skin (discoid lupus erythematous) –> heal and recur –>healing leads to cutaneous atrophy, scarring and altered pigmentation
What do oral lesions associated with chronic cutaneous lupus erythematosus resemble?
Lichen planus
How is lupus erythematous diagnosed?
Through clinical, microscopic features and immunological studies
What are the immunological studies for lupus erythematous?
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]
How is systemic lupus erythematosus treated?
Avoid excessive sunlight
mild = NSAIDs and antimalarials (hydoxychlorquine)
Severe = systemic corticosteroids and immunosuppressive agents
How is chronic cutaneous lupus erythematosus treated?
Avoid excessive sunlight, treat skin lesions with topical corticosteroids
What is the prognosis of systemic lupus erythematosus?
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
What is the prognosis of chronic cutaneous lupus erythematosus?
50% of cases resolve spontaneously
5% progress to systemic lupus erytheamtosus
What term describes a disease in which dense collagen is deposited in the tissues of the body in the extraordinary amounts?
Systemic sclerosis
Who does systemic sclerosis commonly affect?
Females (5:1), aduls
What is the first sign of systemic sclerosis?
Raynaud’s phenomenon - vasoconstrictive event triggered by emotional event or exposure to colde (*this is not specific for systemic sclerosis)
What are some other signs of systemic sclerosis?
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
What is the most serious concern of systemic sclerosis?
Involvement of organs! Fibrosis of lung, heart, kindey, GI tract –> failure within 3 years
What is the primary cause of death for patients with systemic sclerosis?
Pulmonary fibrosis leading to pulmonary HTN and heart failure
What is microstomia and how is it related to systemic sclerosis?
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
What term describes a mild variant of systemic sclerosis and what is it?
Localized scleroderma, and it affects a solitary patch of skin –> looks like scar (en coup de sabre)
How is systemic sclerosis managed?
Hard to manage b/c condition wasex and wanes
Dental appliances for microstomia
Poor prognosis - 10 year survival = 60%
What is CREST syndrome?
Calcinosis cutis, Raynaud’s phenomenon, Esophageal dysfunction, Sclerodactyly, Telangiaectasia
Who does CREST syndrome most commonly affect?
Women in their 60s
What is calcinosis cutis?
Multiple, movable, nontender, subcutaneous, nodular calcifications
What is raynaud’s phenomenon? (CREST)
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
Why does esophageal dysfunction occur with CREST syndrome?
Caused by abnormal collagen deposition in the esophageal submucosa
What is sclerodactyly? (CREST)
Fingers become stiff, skin becomes smooth and shiny –> permanent flexure due to abnormal deposition of collagen within the dermis
What is telangiectasias? (CREST)
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
What is the treatment of CREST syndrome?
Same as systemic sclerosis, and patients should be monitored for pulmonary hypertension (usually will occur 10 years after dx)
What term describes an acquired dermatologic problem with velvety, brownish alterations of the skin?
Acanthosis Nigricans
What can acanthosis nigricans be seen in conjunction with?
Gastrointestinal cancer in 20%, acanthosis associated with these are termed malignant acanthosis
What may cause acanthosis nigricans?
Obesity (psuedoacanthosis nigricans), inheritance, various endocrinopathies (DM), various syndromes (Crouzon), various drugs (contraceptives)
Where does both benign and malignant acanthosis nigricans occur?
The flexural areas of the skin
Can oral lesions occur with acanthosis nigricans?
Yes, in up to 50% and especially in those with the malignant form
What do oral lesions of acanthosis nigricans appear as?
Diffuse, finely papillary areas of mucosal alteration that most often involve the tongue or lips (esp. upper lip) but usually not brown
What is the treatment of acanthosis nigricans?
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
