Immune-Mediated Mucocutaneous 3 & 4 Flashcards
T/F: Cutaneous Lichen Planus is a CHRONIC disorder
true
- typically disease course is 1-2 years
what groups are most likely to develop Cutaneous Lichen Planus?
Affects adults (1%), 30-60 years of age
Female predilection
what are the clinical characteristics of cutaneous lichen planus?
A) Purple pruritic polygonal papules
B) Wickham’s striae
where are Cutaneous Lichen Planus lesions typically found?
how long does this disease typically last?
Flexor surface of wrists, lumbar region, shins
Typically, 1-2 yr disease course
T/F: Oral Lichen Planus only occurs in association with Cutaneous Lichen Planus
False
May occur alone or with skin lesions
what groups are most likely to develop Oral Lichen Planus?
Adults, with female predilection (3:2)
Same as cutaneous
where in the mouth is Oral Lichen Planus found?
which form of oral lichen planus is more COMMON?
which form is more SYMPTOMATIC?
Bilateral buccal mucosa, tongue, gingiva
Reticular form is most common
Erosive form is most symptomatic
Which condition has a longer disease course: oral, or cutaneous, Lichen Planus
ORAL Lichen planus lasts longer
- Protracted disease course, years-decades
what do the lesions from RETICULAR oral lichen planus look like?
Reticular LP: interlacing white lines
white color due to hyperkeratosis
where in the mouth would Reticular Oral Lichen Planus show patchy keratosis and atrophy?
Dorsal tongue
what are the clinical manifestations of EROSIVE Oral Lichen planus?
shallow ulcers, peripheral erythema and radiating white lines
T/F: C. albicans infections may be superimposed on BOTH Reticular and Erosive forms of oral lichen planus
true
what are the main histopathological finding for Oral Lichen Planus?
1) Hyperkeratosis
2) Alternating atrophy/thickening of spinous layer
3) pointed rete ridges
4) degeneration of the basal cell layer
5) band-like infiltrate of lymphocytes
How is Oral Lichen Planus treated?
give treatment for BOTH reticular and erosive forms
A) Management includes treatment of any associated candidiasis
B) Reticular LP: usually requires no therapy
C) Erosive LP: should be treated with one of the stronger topical corticosteroids; systemic steroids usually not needed
what is the prognosis for oral lichen planus?
Is OLP precancerous?
A) Prognosis is good
- Controversy exists regarding the OLP as premalignant condition
- Current best molecular evidence does not support a premalignant potential
Oral lichen planus can be confused with what other broad disease category?
Lichenoid Mucositis
what are some types of Lichenoid Mucositis?
A) Lichenoid drug reaction
B) lichenoid amalgam reaction
C) oral mucosal cinnamon reaction
D) lichenoid foreign body gingivitis
E) oral lesions of GVHD or lupus erythematous
F) some epithelial dysplasias
Which disease is “The most common of the significant immune-mediated systemic diseases”
Systemic Lupus Erythematosus
How many Americans are affected by Systemic Lupus Erythematosus?
What population groups are at highest risk?
Approximately 1.5 million affected in U.S.
Women affected 8 to 10 times more often than men
Average age at diagnosis – 31 years
what are the INITIAL symptoms of Systemic Lupus Erythematosus?
Protean (variable) manifestations initially:
1) Fever, weight loss, arthritis, fatigue, malaise
2) Malar “butterfly” rash
3) Skin lesions flare with sun exposure
what are the effects of Systemic Lupus Erythematosus on the organ systems?
1) Renal involvement (40-50%), renal failure
2) Cardiac involvement is common, particularly pericarditis
3) 50% have vegetations on heart valves (Libman-Sacks endocarditis)
What is often the most significant clinical complication for Systemic Lupus Erythematosus (SLE)?
renal failure
Oral lesions are present in _______% of SLE
5-25%
what are the characteristics of SLE oral lesions?
- May appear as non-specific or lichenoid ulcerations/keratotic areas
- Affect palate, buccal mucosa, or gingiva
T/F: the treatment for SLE oral lesions is usually topical corticosteroids
true
- secondary candidiasis infection is common
what are the microscopic characteristics of Systemic Lupus Erythematosus (SLE)?
A) Lichenoid pattern microscopically
B) Positive lupus band test – deposition of immunoreactants at BMZ of normal skin
C) Anti-nuclear antibodies present (95%), especially to dsDNA
how is SLE treated?
Treatment includes:
A) decreasing patient’s exposure to UV light
B) Mild disease- NSAID agents or anti-malarial drugs
C) more significant cases- Systemic corticosteroids
what conditions would warrant the use of Systemic corticosteroids to treat SLE?
- arthritis
- pericarditis
- nephritis
- thrombocytopenia
what is the prognosis for SLE?
what about the 5 and 10-year survival rates?
Prognosis is variable
5-year survival is 95%
20-year survival falls to 75%
Do men or women have a worse prognosis for SLE?
Worse for MEN
more common in women, hits men harder
_________________ is related to SLE, but almost exclusively affects skin and mucosa
Chronic Cutaneous Lupus
what is the clinical presentation for Chronic Cutaneous Lupus?
Scaly, erythematous patches appear on sun-exposed skin of the head and neck
Scarring and atrophy as these lesions heal
what do the ORAL lesions of CCL resemble?
Oral lesions resemble erosive Lichen Planus
T/F: The clinical appearance of CCL skin lesions usually is characteristic
true
what are the microscopic features of CCL lesions?
Microscopic examination of oral lesion shows lichenoid mucositis and vasculitis
Which form of lupus typically presents with anti-nuclear antibodies? which does not?
SLE = anti-nuclear antibodies
CCL = typically none
How is CCL treated?
A) Treatment usually includes avoiding excessive UV light exposure
B) Topical corticosteroids or calcineurin inhibitors are often helpful with either cutaneous or oral lesions
C) More difficult cases may require systemic anti-malarial drug therapy or low-dose thalidomide
T/F: the prognosis for Chronic Cutaneous Lupus is much better than for Systemic lupus
true
Approximately ___% of CCLE patients may transform to SLE
5
what % of Chronic Cutaneous Lesions will resolve after several years?
50%
________________ is also known as “Scleroderma”
Systemic Sclerosis
what causes Systemic Sclerosis?
inappropriate deposition of dense collagen
what is the prevalence of Systemic Sclerosis?
10-20 cases/million
what groups are at highest risk for developing systemic sclerosis?
Women affected 3-5 times more frequently than men
adult age group
**same as all the others in this section
what are the clinical characteristic features of Systemic Sclerosis?
(NOT the oral characteristics)
A) Raynaud’s phenomenon; claw-like deformity of fingers; acro-osteolysis
B) Diffuse smooth, hard texture of skin
what organ systems can be effected by Systemic Sclerosis?
Pulmonary, renal, cardiac and gastrointestinal FIBROSIS may be seen
— Pulmonary fibrosis leads to hypertension and heart failure
what are the ORAL characteristics of Systemic Sclerosis?
1) Microstomia, with pinched, “purse-string” appearance of mouth
2) Dysphagia with esophageal involvement
3) Diffuse widening of periodontal ligament
4) Resorption of posterior ramus, condyle, coronoid process or chin in 10-20%
what antibodies are present during Systemic Sclerosis?
A) Serologic studies show autoantibodies directed against Scl-70 (topoisomerase 1)
B) Anticentromere antibodies
Anticentromere antibodies are associated more with ______ syndrome than with Systemic Sclerosis
associated more with CREST syndrome
what are some possible treatment options for Systemic Sclerosis?
- No uniformly effective treatment -
Supportive care includes:
esophageal dilation, calcium channel blockers to ease Raynaud’s symptoms, ACE inhibitors to control hypertension
what is the prognosis for both limited & diffuse Systemic Sclerosis?
10 year survival:
- 75-80% with limited disease
- 55-60% with diffuse disease
_____________ is a mild variant of Systemic Sclerosis
CREST Syndrome
what does “CREST” stand for?
Calcinosis cutis Raynaud’s phenomenon Esophageal dysfunction Sclerodactyly Telangiectasia
what population group is most likely to develop CREST syndrome?
Women
6th to 7th decade of life
how is CREST syndrome treated?
how does the prognosis compare to Systemic Sclerosis?
A) Symptomatic care, similar to systemic sclerosis
B) Better prognosis than systemic sclerosis
what causes Libman-Sacks endocarditis?
Systemic Lupus erythematous
