Jones 2 Flashcards

(33 cards)

1
Q

<p>Features of Lupus Erythematosus</p>

A

<p>-Immunologically mediated, multisystem disease

- Type III hypersensitivity reaction
- Three forms of the disease
- Systemic Lupus Erythematosus
- Chronic Cutaneous Lupus Erythematosus
- Subacute Cutaneous Lupus Erythematosus</p>

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2
Q

<p>Features of Systemic Lupus Erythematosus</p>

A

<p>-F > M; average age at diagnosis = 31

- Fever, weight loss, arthritis, fatigue, malaise
- Butterfly rash over the malar area and nose
- Kidney failure and cardiac involvement
- Oral lesions develop in 5-25% of patients</p>

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3
Q

<p>Features of Chronic Cutaneous Lupus Erythematosus</p>

A

<p>-Few or no systemic signs or symptoms

- Lesions limited to the skin or mucosal surfaces
- Skin lesions: discoid lupus erythematosus
- Oral lesions: similar to those seen in erosive lichen planus</p>

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4
Q

<p>Features of Subacute Cutaneous Lupus Erythematosus</p>

A

<p>-Clinical manifestations are intermediate between those of SLE and CCLE

- Skin lesions are more prominent than mucosal lesions
- Most patients have arthritis or musculoskeletal problems</p>

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5
Q

<p>Histopathologic features of Lupus Erythematosus</p>

A

<p>-Hyperkeratosis

- Alternating atrophy and thickening of the spinous cell layer
- Degeneration of the basal cell layer
- Subepithelial lymphocytic infiltrate, often in a perivascular pattern
- Deposition of eosinophilic material in basement membrane zone; subepithelial edema</p>

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6
Q

<p>Diagnosis of Lupus Erythematosus</p>

A

<p>-DIF of lesional tissue: shaggy or granular band of IgM, IgG, or C3 at the basement membrane zone

- DIF of normal tissue: similar deposition of IgM, IgG, or C3
- Antibodies against double-stranded DNA (70%)
- Antibodies against Sm - small nuclear RNA (30%)</p>

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7
Q

<p>Treatment and prognosis of Lupus Erythematosus</p>

A

<p>-Avoid exposure to ultraviolet light

- Corticosteroids and immunosuppressive agents
- SLE: 5-year survival rate = 95%, 15-year survival rate = 75%</p>

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8
Q

<p>Features of Lichen Planus</p>

A

<p>-Common, chronic dermatologic disease that often affects the oral mucosa

- Represents a T-cell mediated immunologic process (Type IV hypersensitivity reaction)
- Middle-aged adults; F > M</p>

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9
Q

<p>Features of Cutaneous Lichen Planus</p>

A

<p>-Purple, pruritic, polygonal papules

- Flexor surfaces of the extremities
- Lacelike network of white lines (Wickham striae)
- 1% of the population has cutaneous lichen planus</p>

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10
Q

<p>Features of Oral Lichen Planus</p>

A

<p>-3 forms: Reticular, Erosive, Desquamative gingivitis

- Bilaterally symmetrical
- Most common location: buccal mucosa
- 0.1-2.2% of the population has oral lichen planus</p>

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11
Q

<p>Histopathologic features of Lichen Planus</p>

A

<p>-Orthokeratosis or parakeratosis

- "Saw toothed" rete pegs
- Hydropic degeneration of the basal cell layer
- Colloid, cytoid, hyaline, or Civatte bodies
- Band-like infiltrate of T lymphocytes in the superficial connective tissue
- DIF: linear band of fibrinogen at the basement membrane</p>

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12
Q

<p>Treatment and prognosis of Lichen Planus</p>

A

<p>-Corticosteroids

| -Questionable malignant transformation</p>

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13
Q

<p>Features of Aphthous Stomatitis</p>

A

<p>-Common ulcerative disease that affects ~20% of the population

- Thought to result from a T-cell mediated immunologic reaction
- Destruction is due to direct T lymphocyte-mediated cytotoxicity</p>

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14
Q

<p>Causative agents of Aphthous Stomatitis</p>

A

<p>-Allergies

- Genetic predisposition
- Nutritional deficiencies
- Hematologic abnormalities
- Hormonal influences
- Infectious agents
- Trauma
- Stress</p>

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15
Q

<p>Clinical variants and prodromal symptoms of Aphthous Stomatitis</p>

A

<p>-Three clinical variants: minor, major, herpetiform

| -Prodromal symptoms may occur: burning, itching, stinging</p>

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16
Q

<p>Features of Minor Aphthous Ulcerations</p>

A

<p>-Ulcers are several millimeters in size

- Single or multiple ulcers may be present
- Occur on moveable mucosa
- Any age; F > M
- Heal without scarring in 7-14 days</p>

17
Q

<p>Features of Major Aphthous Ulcerations</p>

A

<p>-Ulcers are usually 1-3 centimeters in size

- Single or multiple ulcers may be present
- Occur on moveable mucosa
- Typically arise after puberty
- Heal in 2-6 weeks
- Heal with scarring</p>

18
Q

<p>Features of Herpetiform Aphthous Ulcerations</p>

A

<p>-Multiple ulcers are present (>100)

- Ulcers are 1-3 millimeters in size
- Occur on moveable or fixed mucosa
- Arise in adults; F > M
- Heal in 7-10 days, but recurrence is common</p>

19
Q

<p>Diagnosis and treatment of Aphthous Stomatitis</p>

A

<p>-Diagnosis is usually made on clinical presentation

- Biopsy may be performed
- Treatment: topical corticosteroids
- Recurrent disease: evaluate for an underlying systemic disease</p>

20
Q

<p>Features of Sarcoidosis</p>

A

<p>-Multisystem granulomatous disorder of unknown cause

- Blacks > white; F > M; 20-40 years of age
- Any organ may be affected but pulmonary symptoms are most common followed by lymph nodes, skin, eyes, and salivary glands
- Bilateral hilar lymphadenopathy - 90% of patients</p>

21
Q

<p>Skin manifestations of Sarcoidosis</p>

A

<p>-Occur in 25% of patients

- Lupus pernio: violaceous, indurated lesions
- Erythema nodosum: erythematous nodules</p>

22
Q

<p>Other involvements of Sarcoidosis</p>

A

<p>-Ocular involvement: 25% of patients, anterior uveitis, keratoconjunctivitis
-Salivary gland involvement: enlargement, xerostomia</p>

23
Q

<p>Oral manifestations of Sarcoidosis</p>

A

<p>-Uncommon

- Any mucosal site
- Submucosal mass or papule
- Ill-defined radiolucency in bone</p>

24
Q

<p>Syndromes associated with Sarcoidosis</p>

A

<p>-Lofgren syndrome: Erythema nodosum, bilateral hilar lymphadenopathy, arthralgia
-Heerfordt syndrome: Parotid enlargement, anterior uveitis, facial paralysis, fever</p>

25

Histopathologic features of Sarcoidosis

-Granulomatous inflammation - Schaumann bodies - basophilic calcifications - Asteroid bodes - stellate inclusions

26

Diagnosis of Sarcoidosis

-Elevated serum angiotensin-converting enzyme (ACE) - Kveim test: intradermal injection of a sterilized suspension of human sarcoid tissue - papule develops 4-6 weeks later in affected patients - Salivary gland biopsy

27

Treatment of Sarcoidosis

-In 60% of patients, symptoms resolve spontaneously - Corticosteroids - 4-10% die of pulmonary, cardiac, or CNS complications

28
Features of Wegener Granulomatosis
- Uncommon disease of unknown cause - Wide age range; M = F - Any organ may be involved: 1. Necrotizing granulomas of the respiratory tract 2. Necrotizing glomerulonephritis 3. Systemic vasculitis of small arteries and veins
29
Common signs and symptoms of Wegener Granulomatosis
- Purulent nasal discharge - Chronic sinus pain, epistaxis - Saddle nose deformity (destruction of the nasal septum) - Cough, hemoptysis, dyspnea - Proteinuria, red cell casts
30
Skin involvement of Wegener Granulomatosis
Vasculitis of small arteries and veins leads to areas of erythema, erosion, and ulceration
31
Oral manifestations of Wegener Granulomatosis
- Strawberry gingivitis - Destruction of underlying bone and tooth mobility - Ulcerations - Major salivary gland enlargement
32
Microscopic features of Wegener Granulomatosis
- Leukocytoclastic vasculitis: Neutrophilic infiltration, necrosis, nuclear dust - Mixed inflammatory infiltrate in adjacent connective tissue - Pseudoepitheliomatous hyperplasia
33
Diagnosis, treatment, and prognosis of Wegener Granulomatosis
- Diagnosis by IIF: antibodies against cytoplasmic components of neutrophils: c-ANCA - positive in 90-95% of cases - Treatment: Cyclophosphamide and prednisone - Prognosis: Variable depending on extent of disease