Fa: Msk, Skin, And Ct - Pathology

Front 1

What diagnosis do you suspect in a patient with short limbs and normal sized head and torso (large relative to limbs)?

Back 1

Achondroplasia

Front 2

What type of bone growth is compromised in Achondroplasia? Why?

Back 2

- Failure of longitudinal bone growth (endochondral ossification)
- Membranous ossification is not affected
- This is due to constitutive activation of fibroblast growth factor receptor (FGFR3)

Front 3

What causes Achondroplasia?

Back 3

- Constitutive activation of fibroblast growth factor receptor (FGFR3)
- Inhibits chondrocyte proliferation
- 85% of mutations occur sporadically and associated with advanced paternal age
- Also associated with autosomal dominant inheritance

Front 4

How are the life span and fertility of a patient with Achondroplasia affected?

Back 4

Normal life span and fertility

Front 5

What type of bone is affected by Osteoporosis? What happens to it?

Back 5

Trabecular (spongy) bone loses mass and interconnections, despite normal bone mineralization and lab values (serum Ca2+ and PO4(3-))

Front 6

What are the typical lab values for serum Ca2+ and PO4(3-) in patients with Osteoporosis?

Back 6

Ca2+ and PO4(3-) are normal

Front 7

How do you diagnose Osteoporosis?

Back 7

Bone Mineral Density test (DEXA scan) with a T-score of ≤ -2.5

Front 8

What can cause Osteoporosis?

Back 8

- Long-term exogenous steroid use
- Menopause: ↓ estrogen
- Senile (>70 years)

Front 9

What are some of the potential consequences of Osteoporosis?

Back 9

Vertebral crush fractures:
- Acute back pain
- Loss of height
- Kyphosis

Front 10

What are the two types of Osteoporosis?

Back 10

- Type 1: post-menopausal
- Type 2: senile (men and women > 70 years)

Front 11

What causes Type 1 Osteoporosis? Complications?

Back 11

- Post-menopausal: ↑ bone resorption due to ↓ estrogen levels
- Complications: femoral neck fracture, distal radius (Colles) fracture

Front 12

What causes Type 2 Osteoporosis? Treatment?

Back 12

- Senile osteoporosis: affects men and women >70 years old
- Prophylaxis: regular weight-bearing exercise and adequate Ca2+ and Vitamin D intake throughout adulthood
- Treatment: bisphosphanates, PTH, SERMs, rarely calcitonin; denosumab (monoclonal Ab against RANKL)

Front 13

Which disease is also known as Marble Bone Disease?

Back 13

Osteopetrosis

Front 14

What is wrong in Osteopetrosis?

Back 14

Failure of normal bone resorption due to defective osteoclasts → thickened dense bones that are prone to fracture

Front 15

What is the name for thickened bones due to failure of bone resorption? Consequences?

Back 15

Osteopetrosis (marble bone disease)
- Prone to fracture
- Pancytopenia and extramedullary hematopoiesis (bone fills BM space)
- Cranial nerve impingement and palsies (narrowed foramina)

Front 16

What can cause Osteopetrosis?

Back 16

Mutations (eg, carbonic anhydrase II) impair ability of osteoclast to generate acidic environment necessary for bone resorption

Front 17

What is the appearance of Osteopetrosis on an x-ray?

Back 17

- Bone-in-bone appearance
- Thickened, dense bones

Front 18

How do you treat a patient with Osteopetrosis?

Back 18

Bone marrow transplant is potentially curative because osteoclasts are derived from monocytes; the problem is defective osteoclasts

Front 19

What are the names of the diseases caused by a Vitamin D deficiency?

Back 19

- Osteomalacia in adults
- Rickets in children

Front 20

What causes osteomalacia (adults) and rickets (children)? Implications?

Back 20

Vitamin D deficiency
- Defective mineralization / calcification of osteoid → soft bones that bow out
- ↓ Vitamin D → ↓ serum Ca2+ → ↑ PTH → ↓ serum PO4(3-)
- Hyperactivity of osteoblasts → ↑ ALP (osteoblasts require alkaline environment)

Front 21

What is the implication of osteoblast hyperactivity in Osteomalacia / Rickets?

Back 21

Increased Alkaline Phosphatase (ALP) because osteoblasts require an alkaline environment

Front 22

What disorder is characterized by increase in both osteoblastic and osteoclastic activity?

Back 22

Paget Disease of Bone (Osteitis Deformans)

Front 23

What are labs found in Paget Disease of Bone (Osteitis Deformans)?

Back 23

- Serum Ca2+, Phosphorus, and PTH are all normal
- ↑ ALP

Front 24

What is the appearance of bone in Paget Disease of Bone (Osteitis Deformans)?

Back 24

Mosaic pattern of women and lamellar bone

Front 25

What are the potential complications of Paget Disease of Bone (Osteitis Deformans)?

Back 25

- Long bone chalk-stick fractures
- ↑ Blood flow from ↑ AV shunts may cause high-output heart failure
- ↑ Risk of Osteogenic Sarcoma
- Hearing loss due to auditory foramen narrowing

Front 26

In what disease is there a characteristic "increase in hat size"? Why?

Back 26

Paget Disease of Bone (Osteitis Deformans)
- Marked thickening of calvarium

Front 27

What are the stages of Paget Disease? Which cells are active during these stages?

Back 27

- Lytic: osteoclasts
- Mixed: osteoclasts and osteoblasts
- Sclerotic: osteoblasts
- Quiescent: minimal osteoclast/osteoblast activity

Front 28

What can cause Osteonecrosis (avascular necrosis)?

Back 28

Infarction of bone and marrow
- Trauma
- High dose corticosteroids
- Alcoholism
- Sickle cell disease

Front 29

What happens in Osteonecrosis? Most common location? Why?

Back 29

- Infarction of bone and marrow, usually very painful
- Most common site is femoral head (d/t insufficiency of medial circumflex femoral artery)

Front 30

In which bone disorders are the serum Ca2+, PO4(3-), ALP, and PTH all normal? Implications?

Back 30

- Osteoporosis → ↓ bone mass
- Osteopetrosis → dense, brittle bones; Ca2+ ↓ in severe malignant disease

Front 31

In which bone disorders are the serum Ca2+, PO4(3-), and PTH all normal, but with an elevated ALP? Implications?

Back 31

Paget Disease → abnormal mosaic bone architecture

Front 32

In which bone disorders are the serum Ca2+ and PO4(3-) decreased, but the ALP and PTH are elevated? Implications?

Back 32

Osteomalacia / Rickets → soft bones

Front 33

In which bone disorders are the serum Ca2+ and PO4(3-) increased, with a normal ALP, and decreased PTH? Implications?

Back 33

Hypervitaminosis D (caused by over-supplementation or granulomatous disease, such as sarcoidosis)

Front 34

In which bone disorders are the ALP and PTH elevated, with opposite levels of Ca2+ and PO4(3-)?

Back 34

Osteitis Fibrosa Cystica
- 1° Hyperparathyroidism: ↑ Ca2+ and ↓ PO4(3-)
- 2° Hyperparathyroidism: ↓ Ca2+ and ↑ PO4(3-)

Front 35

What are "brown tumors"?

Back 35

- Bone lesion that arises in settings of excess osteoclast activity, such as hyperparathyroidism
- It is not a true neoplasm, as the term "tumor" suggests; but, it may mimic a true neoplasm
- Exhibits FIBROUS REPLACEMENT of bone, with SUBPERIOSTEAL THINNING

Front 36

What are causes of 1° hyperparathyroidism?

Back 36

- Idiopathic or parathyroid hyperplasia
- Parathyroid adenoma
- Parathyroid carcinoma

Front 37

2° hyperparathyroidism is often a compensation for what?

Back 37

End-Stage Renal Disease / ESRD
- ↓ PO4(3-) excretion and production of activated vitamin D

Front 38

What are the types of benign primary bone tumors?

Back 38

- Giant cell tumor
- Osteochondroma (exostosis)

Front 39

What is the most common benign bone tumor? Who is most likely to get this type of tumor?

Back 39

Osteochondroma - males <25 years old especially

Front 40

What type of bone tumor should you expect in a young patient, if present in the epiphyseal end of long bones, with a "soap bubble" appearance on x-ray? Characteristics?

Back 40

Giant Cell Tumor
- Benign primary bone tumor
- Locally aggressive, often around the knee
- Multi-nucleated giant cells

Front 41

What type of bone tumor should you expect in a male <25 years that appears as an outgrowth from mature bone with a cartilaginous cap? Characteristics?

Back 41

Osteochondroma (Exostosis)
- Most common benign tumor
- Rarely transforms to chondrosarcoma

Front 42

What are the types of malignant primary bone tumors?

Back 42

- Osteosarcoma (osteogenic sarcoma)
- Ewing sarcoma
- Chondrosarcoma
(Multiple Myeloma)

Front 43

What are the most common 1° malignant bone tumors?

Back 43

1. Multiple Myeloma
2. Osteosarcoma (Osteogenic Sarcoma)

Front 44

What are the most likely malignant bone cancers by age?

Back 44

- Boy < 15 years: Ewing Sarcoma
- 10-20 years: Osteosarcoma (1°)
- 30-60 years: Chondrosarcoma
- >65 years: Osteosarcoma (2°)

Front 45

What are the most common locations for the different malignant bone tumors?

Back 45

- Osteosarcoma: metaphysis of long bones, often around knee
- Ewing Sarcoma: diaphysis of long bones, pelvis, scapula, ribs
- Chondrosarcoma: pelvis, spine, scapula, humerus, tibia, or femur

Front 46

Which type of bone pathology is associated with an elevation of the periosterum? What is the term for this?

Back 46

Osteosarcoma
- Elevation of the periosterum = Codman Triangle

Front 47

Which type of bone pathology is associated with a "sunburst pattern" on x-ray?

Back 47

Osteosarcoma

Front 48

What are the predisposing factors for Osteosarcoma?

Back 48

- Paget disease of bone
- Bone infarcts
- Radiation
- Familial retinoblastoma
- Li-Fraumeni syndrome (germline p53 mutation)

Front 49

How do you treat Osteosarcoma?

Back 49

Surgical en block resection (with limb salvage) and chemotherapy

Front 50

Which bone pathology has an "onion skin" appearance?

Back 50

Ewing Sarcoma

Front 51

Which bone pathology is associated with anaplastic, malignant small blue cells?

Back 51

Ewing Sarcoma

Front 52

Which genetic change is associated with Ewing Sarcoma?

Back 52

t(11;22) translocation

Front 53

How do you treat Ewing Sarcoma?

Back 53

Chemotherapy (responsive)

Front 54

Which bone pathology typically appears as an expansile glistening mass within the medullary cavity?

Back 54

Chondrosarcoma

Front 55

What are the characteristics of Osteosarcoma (when is it more common? predisposing factors? location? signs on labs/imaging? treatment?)

Back 55

- 2nd most common 1° malignant bone tumor
- Bimodal distribution: 10-20 years (1°) and >65 years (2°)
- Predisposing factors: Paget disease of bone, bone infarcts, radiation, familial retinoblastoma, Li-Fraumeni syndrome (germline p53 mutation)
- Location: metaphysis of long bones, often around knee
- Signs: Codman triangle (from elevation of periosteum) or sunburnt pattern on x-ray
- Treatment: aggressive, treat w/ surgical en bloc resection (w/ limb salvage) and chemotherapy

Front 56

What are the characteristics of Ewing Sarcoma (when is it more common? predisposing factors? location? signs on labs/imaging? treatment? other?)

Back 56

- Boys < 15 years old
- Predisposing factors: t(11;22) translocation
- Location: diaphysis of long bones, pelvis, scapula, ribs
- Signs: anaplastic small blue cell malignant tumor, "onion skin" appearance in bone
- Treatment: responsive to chemotherapy
- Extremely aggressive w/ early metastases

Front 57

What are the characteristics of Chondrosarcoma (when is it more common? location? signs on labs/imaging? other?)

Back 57

- Men 30-60 years old
- Location: usually in pelvis, spine, scapula, humerus, tibia, femur
- Signs: expansile glistening mass within the medullary cavity
- Rare, malignant, cartilaginous tumor
- May be 1° origin or from osteochondroma

Front 58

What is the cause of Osteoarthritis vs Rheumatoid Arthritis?

Back 58

- OA: mechanical - joint wear and tear destroys articular cartilage
- RA: auto-immune - inflammatory destruction of synovial joints; mediated by cytokines and type III and IV hypersensitivity reactions

Front 59

What are the findings in joints with Osteoarthritis vs Rheumatoid Arthritis?

Back 59

- OA: subchondral cysts, sclerosis, osteophytes (bone spurs), eburnation (polished, ivory-like appearance), Heberden nodes (DIP), and Bouchard nodes (PIP); no MCP involvement
- RA: pannus formation in joints (MCP and PIP); subcutaneous rheumatoid nodules (fibrinoid necrosis), ulnar deviation of fingers, subluxation, Baker cyst (in popliteal fossa); no DIP involvement

Front 60

What joints are classically affected by Osteoarthritis vs Rheumatoid Arthritis?

Back 60

- OA: DIP and PIP (no MCP); weight-bearing joints
- RA: MCP and PIP (no DIP)

Front 61

What are the predisposing factors for Osteoarthritis vs Rheumatoid Arthritis?

Back 61

- OA: age, obesity, joint deformity, trauma
- RA: female, 80% are + for rheumatoid factor (anti-IgG Ab); anti-cyclic citrullinated peptide Ab is more specific; strongly associated with HLA-DR4

Front 62

What is the classic presentation of Osteoarthritis vs Rheumatoid Arthritis?

Back 62

- OA: pain in weight-bearing joints after use (eg, at end of day), improves with rest; knee cartilage loss begins medially (bowlegged); non-inflammatory; no systemic symptoms
- RA: morning stiffness >30 minutes improves with use; symmetric joint involvement; systemic symptoms (fever, fatigue, pleuritis, and pericarditis)

Front 63

What is the treatment for Osteoarthritis vs Rheumatoid Arthritis?

Back 63

- OA: NSAIDs, intra-articular glucocorticoids
- RA: NSAIDs, glucocorticoids, disease-modifying agents (methotrexate, sulfasalazine, TNF-α inhibitors)

Front 64

Which type of bone pathology is associated with subchondral cysts?

Back 64

Osteoarthritis

Front 65

Which type of bone pathology is associated with sclerosis?

Back 65

Osteoarthritis

Front 66

Which type of bone pathology is associated with pannus formation in the joints (MCP, PIP)?

Back 66

Rheumatoid Arthritis

Front 67

Which type of bone pathology is associated with osteophytes (bone spurs)?

Back 67

Osteoarthritis

Front 68

Which type of bone pathology is associated with ulnar deviation of the fingers?

Back 68

Rheumatoid Arthritis

Front 69

Which type of bone pathology is associated with subluxation?

Back 69

Rheumatoid Arthritis

Front 70

Which type of bone pathology is associated with eburnation? What does this term mean?

Back 70

Osteoarthritis - eburnation means polished, ivory-like appearance of the bone

Front 71

Which type of bone pathology is associated with Baker cysts? Location?

Back 71

Rheumatoid Arthritis - Baker cysts in popliteal fossa

Front 72

Which type of bone pathology is associated with Heberden nodes and Bouchard nodes? Location?

Back 72

Osteoarthritis
- Heberden nodes: DIP
- Bouchard nodes: PIP

Front 73

What type of antibodies are associated with Rheumatoid Arthritis? Which is more specific?

Back 73

- Rheumatoid Factor (anti-IgG antibody)
- Anti-cyclic citrullinated peptide antibody (more specific)

Front 74

What HLA is Rheumatoid Arthritis associated with?

Back 74

HLA-DR4

Front 75

Which type of arthritis has pain in the weight bearing joints after use (eg, at the end of the day)?

Back 75

Osteoarthritis

Front 76

Which type of arthritis has morning stiffness lasting >30 minutes that improves with use?

Back 76

Rheumatoid Arthritis

Front 77

Which type of arthritis has cartilage loss that begins medially?

Back 77

Osteoarthritis

Front 78

How do you treat Osteoarthritis?

Back 78

- NSAIDs
- Intra-articular glucocorticoids

Front 79

How do you treat Rheumatoid Arthritis?

Back 79

- NSAIDs
- Glucocorticoids
- Disease-modifying agents: methotrexate, sulfasalazine, TNF-α inhibitors

Front 80

What type of arthritis has these features?

Back 80

Osteoarthritis

Front 81

What type of arthritis has these features?

Back 81

Rheumatoid Arthritis

Front 82

What type of arthritis has this appearance?

Back 82

Osteoarthritis
- X-rays of the knee show joint space narrowing and sclerosis

Front 83

What type of arthritis has this appearance?

Back 83

Rheumatoid Arthritis
- Note boutonnière deformities of PIP joints with ulnar deviation

Front 84

What disorder causes destruction of exocrine glands via an auto-immune process? Which glands are especially affected?

Back 84

Sjögren Syndrome
- Especially affects lacrimal and salivary

Front 85

Which disorder causes xerophthalmia, xerostomia, and bilateral parotid gland enlargement? What do these terms mean?

Back 85

Sjögren Syndrome
- Xerophthalmia: ↓ tear production and subsequent corneal damage
- Xerostomia: ↓ saliva production

Front 86

What is the term for decreased tear production and subsequent corneal damage?

Back 86

Xerophthalmia

Front 87

What is the term for decreased saliva production?

Back 87

Xerostomia

Front 88

What antibodies are seen in Sjögren Syndrome?

Back 88

Anti-nuclear antibodies: SS-A (anti-Ro) and/or SS-B (anti-La)

Front 89

What can cause Sjögren Syndrome?

Back 89

- Can be primary
- Can be secondary, associate with other auto-immune disorders (eg, rheumatoid arthritis)

Front 90

What are the potential consequences of Sjögren Syndrome?

Back 90

- Dental caries
- Mucosa-associated lymphoid tissue (MALT) lymphoma (may present as unilateral parotid enlargement)

Front 91

What are the findings of Sjögren Syndrome?

Back 91

- Xerophthalmia (↓ tear production and subsequent corneal damage)
- Xerostomia (↓ saliva production)
- Presence of anti-nuclear antibodies: SS-A (anti-Ro) and/or SS-B (anti-La)
- Bilateral parotid enlargement

Front 92

What are the characteristic findings of gout?

Back 92

Acute inflammatory monoarthritis

Front 93

What causes gout?

Back 93

Precipitation of monosodium urate crystals in joints

Associated with hyperuricemia, which can be caused by:
- Underexcretion of uric acid (90% of patients) - largely idiopathic, can be exacerbated by certain medications (eg, thiazide diuretics)
- Overproduction of uric acid (10% of patients) - Lesch-Nyhan syndrome, PRPP excess, ↑ cell turnover (eg, tumor lysis syndrome), von Gierke disease

Front 94

What can cause underexcretion of uric acid? How often is this the cause of gout?

Back 94

- Largely idiopathic
- Can be exacerbated by certain medications (eg, thiazide diuretics)
- 90% of patients

Front 95

What can cause overproduction of uric acid? How often is this the cause of gout?

Back 95

- Lesch-Nyhan syndrome
- PRPP excess
- ↑ Cell turnover (eg, tumor lysis syndrome)
- von Gierke disease
- 10% of patients

Front 96

What is the characteristic of the monosodium urate crystals found in gout?

Back 96

- Needle shaped
- Negatively birefringent (yellow under parallel light, blue under perpindicular light)

Front 97

How does gout typically present?

Back 97

- Asymmetric joint distribution (typically a monoarthritis)
- Joint is swollen, red, and painful
- Classically in the MTP joint of the big toe (podagra)
- Tophus formation
- Acute attacks tend to occur after a large meal or alcohol consumption

Front 98

What are tophi? Where do they form?

Back 98

Tophus (deposit of crystalline uric acid and other substances at the surface of joints or in skin or cartilage)
- Often on external ear, olecranon bursa, or Achilles tendon

Front 99

When do acute attacks of gout often occur? Why?

Back 99

- After a large meal
- After alcohol consumption: alcohol metabolites compete for same excretion sites in kidney as uric acid, causing ↓ uric acid secretion and subsequent buildup in blood

Front 100

What are the treatments for acute attacks of gout?

Back 100

- NSAIDs (eg, indomethacin)
- Glucocorticoids
- Colchicine

Front 101

What are the chronic preventive treatments for gout?

Back 101

Xanthine oxidase inhibitors (Allopurinal and Febuxostat)

Front 102

What is the mechanism of Allopurinol and Febuxostat? Uses?

Back 102

Xanthine Oxidase inhibitors - used for chronic / preventative treatment of gout

Front 103

What kind of crystals cause pseudogout?

Back 103

Calcium pyrophosphate crystals - basophilic, rhomboid crystals that are weakly birefringent (blue when parallel to light)

Front 104

What is the typical presentation of pseudogout? Who is more often affected?

Back 104

- Pain and effusion in joint
- Usually affects large joints (classically the knee)

- >50 years old, both sexes affected equally

Front 105

What diseases are associated with pseudogout?

Back 105

- Hemochromatosis
- Hyperparathyroidism
- Hypoparathyroidism

Front 106

How does pseudogout appear on x-ray?

Back 106

Chondrocalcinosis - calcium pyrophosphate cyrstals within joint space

Front 107

How do you treat pseudogout?

Back 107

- NSAIDS for sudden, severe attacks
- Steroids
- Colchicine

Front 108

How do gout and pseudogout compare in terms of the birefringence of their crystals?

Back 108

- Gout: crystals are yellow when parallel to light
- Pseudogout: crystals are blue when parallel to light

Front 109

What are the most common causes of infectious arthritis?

Back 109

- S. aureus
- Streptococcus
- Neisseria gonorrhoeae

Front 110

In a sexually active patient, with asymmetric migratory arthritis, what should you consider as a cause?

Back 110

Neisseria gonorrhoeae: gonococcal arthritis (STD)

Front 111

What are the characteristics of infectious arthritis?

Back 111

Joint is swollen, red, and painful

Front 112

What are the specific signs of Gonococcal Arthritis?

Back 112

STD:
- Synovitis (eg, knee)
- Tenosynovitis (eg, hand)
- Dermatitis (eg, pustules)

Front 113

What is the term for arthritis without rheumatoid factor (anti-IgG Ab)? What is it associated with?

Back 113

Seronegative Spondyloarthropathies:
- Strong association with HLA-B27

Front 114

What are the types of Seronegative Spondyloarthropathies?

Back 114

PAIR:
- Psoriatic arthritis
- Ankylosing spondylitis
- Inflammatory bowel disease
- Reactive arthritis (Reiter syndrome)

Front 115

What are the characteristics of arthritis in a patient with psoriasis?

Back 115

- Joint pain and stiffness
- Asymmetric and patchy involvement
- Dactylitis ("sausage fingers")
- "Pencil-in-cup" deformity on x-ray

Front 116

How common is arthritis in patients with psoriasis?

Back 116

1/3 of patients with psoriasis

Front 117

What is the name of the chronic inflammatory disease of the spine and sacroiliac joints? Implications?

Back 117

Ankylosing Spondylitis
- Ankylosis (stiff spine d/t fusion of joints)
- Uveitis
- Aortic regurgitation

Front 118

In what condition do you see "bamboo spine"? Cause?

Back 118

Ankylosing spondylitis
- Vertebral fusion

Front 119

What are the characteristics of arthritis in patients with Inflammatory Bowel Disease?

Back 119

Crohn disease and ulcerative colitis are often accompanied by ankylosing spondylitis or peripheral arthritis

Front 120

What is the classic triad of findings in Reactive Arthritis (Reiter Syndrome)?

Back 120

"Can't see, can't pee, can't bend my knee":
- Conjunctivitis (and anterior uveitis)
- Urethritis
- Arthritis

Front 121

What is Reactive Arthritis (Reiter Syndrome) in response to?

Back 121

Post-GI (Shigella, Salmonella, Yersinia, Campylobacter) or Chlamydia infections

Front 122

What disease has the classic presentation of rash, joint pain, and fever?

Back 122

Systemic Lupus Erythematosus

Front 123

What mnemonic can help you remember the characteristics of Systemic Lupus Erythematosus?

Back 123

RASH OR PAIN:
- Rash (malar or discoid)
- Arthritis
- Soft tissues / Serositis
- Hematologic disorders (eg, cytopenias)
- Oral / nasopharyngeal ulcers
- Renal disease, Raynaud phenomenon
- Photosensitivity, Positive VDRL/RPR
- Anti-nuclear antibodies
- Immunosuppressants
- Neurologic disorders (eg, seizures, psychosis)

Front 124

What are the common causes of death in patients with Systemic Lupus Erythematosus?

Back 124

- Cardiovascular disease
- Infections
- Renal disease

Front 125

What kind of cardiac disease is associated with Systemic Lupus Erythematosus?

Back 125

Libman-Sacks Endocarditis: wart-like vegetations on boths ides of valve

Front 126

What kind of renal disease is associated with Systemic Lupus Erythematosus?

Back 126

Lupus Nephritis (Type III hypersensitivity reaction):
- Nephritic: diffuse proliferative glomerulonephritis
- Nephrotic: membranous glomerulonephritis

Front 127

What kinds of antibodies are associated with Systemic Lupus Erythematosus?

Back 127

- Anti-nuclear antibodies (ANA)
- Anti-dsDNA antibodies
- Anti-Smith antibodies
- Anti-histone antibodies
- Anti-cardiolipin antibodies

Front 128

What antibody is sensitive, but not specific for Systemic Lupus Erythematosus?

Back 128

Anti-nuclear antibodies (ANA)

Front 129

What antibody is specific and indicates a poor prognosis (renal disease) for Systemic Lupus Erythematosus?

Back 129

Anti-dsDNA antibodies

Front 130

What antibody is specific, but does not offer prognostic information?

Back 130

Anti-Smith antibodies (directed against snRNPs)

Front 131

What antibody is sensitive for drug-induced lupus?

Back 131

Anti-histone antibodies

Front 132

What antibody shows false positives for patients with syphilis and prolongs the PTT in patients with Systemic Lupus Erythematosus? Implications?

Back 132

Anti-Cardiolipin antibody
- Pardoxically prolonged PTT, increases risk for arteriovenous thromboembolism

Front 133

What antibody increases the risk of arteriovenous thromboembolism?

Back 133

Anti-cardiolipin antibodies

Front 134

How is complement affected in Systemic Lupus Erythematosus?

Back 134

Decreased C3, C4, and CH50 due to immune complex formation

Front 135

How do you treat patients with Systemic Lupus Erythematosus?

Back 135

- NSAIDs
- Steroids
- Immunosuppressants
- Hydroxychloroquine

Front 136

What mnemonic can help you remember the characteristics of Systemic Lupus Erythematosus?

Back 136

RASH OR PAIN:
- Rash (malar or discoid)
- Arthritis
- Soft tissues / Serositis
- Hematologic disorders (eg, cytopenias)
- Oral / nasopharyngeal ulcers
- Renal disease, Raynaud phenomenon
- Photosensitivity, Positive VDRL/RPR
- Anti-nuclear antibodies
- Immunosuppressants
- Neurologic disorders (eg, seizures, psychosis)

Front 137

What disease is characterized by immune-mediated, widespread non-caseating granulomas and elevated serum ACE levels?

Back 137

Sarcoidosis

Front 138

If your patient is a black female with enlarged lymph nodes and bilateral hilar adenopathy or reticular opacities incidentally found on CXR, what diagnosis should you consider? Why?

Back 138

Sarcoidosis
- CXR shows bilateral suprahilar adenopathy (arrows) and right upper lung reticular opacity (often incidental findings on CXR)
- Common in black females
- Often asymptomatic except for enlarged lymph nodes

Front 139

What is sarcoidosis associated with?

Back 139

- Restrictive lung disease (interstitial fibrosis)
- Erythema nodosum (inflammation of the fat cells under the skin, resulting in tender red nodules or lumps that are usually seen on both shins)
- Lupus pernio (chronic raised indurated (hardened) lesion of the skin, often purplish)
- Bell palsy
- Epithelioid granulomas
- Uveitis
- Hypercalcemia

Front 140

What are the characteristics of the granulomas in Sarcoidosis?

Back 140

- Non-caseating
- Epithelioid
- Contain Schaumann and asteroid bodies

Front 141

What electrolyte abnormality is associated with sarcoidosis? Cause?

Back 141

Hypercalcemia - due to ↑ 1α-hydroxylase-mediated Vitamin D activation in macrophages

Front 142

How do you treat sarcoidosis?

Back 142

Steroids

Front 143

What are the characteristics of Sarcoidosis?

Back 143

- Immune-mediated, widespread non-caseating granulomas
- Elevated serum ACE levels
- Common in black females
- Often asymptomatic except for enlarged lymph nodes
- Incidental findings on CXR: bilateral hilar adenopathy and/or reticular opacities

Front 144

What diagnosis should you consider in a patient with pain and stiffness in their shoulders and hips, with fever, malaise, and weight loss?

Back 144

Polymyalgia Rheumatica

Front 145

What symptoms occur in patients with Polymyalgia Rheumatica?

Back 145

- Pain and stiffness in shoulders and hips
- Fever, malaise, weight loss
- No muscular weakness

Front 146

Who is more likely to have Polymyalgia Rheumatica?

Back 146

- More common in women >50 years old
- Associated with Temporal (Giant Cell) Arteritis

Front 147

What are the lab findings in a patient with Polymyalgia Rheumatica?

Back 147

- ↑ ESR
- ↑ C-reactive protein
- Normal CK

Front 148

How do you treat a patient with Polymyalgia Rheumatica?

Back 148

Rapid response to low-doe corticosteroids

Front 149

Which disease is most commonly seen in females 20-50 years old with chronic widespread musculoskeletal pain, stiffness, paresthesias, poor sleep, and fatigue? How should you treat?

Back 149

Fibromyalgia
- Regular exercise
- Antidepressants (TCAs, SNRIs)
- Anti-convulsants

Front 150

What are the symptoms of Fibromyalgia?

Back 150

- Chronic, widespread musculoskeletal pain
- Associated with stiffness, paresthesias, poor sleep, and fatigue
- Most commonly in females 20-50 years old

Front 151

How do you treat Fibromyalgia?

Back 151

- Regular exercise
- Antidepressants (TCAs, SNRIs)
- Anti-convulsants

Front 152

What is the cause of progressive symmetric proximal muscle weakness, characterized by endomysial inflammation with CD8+ T cells? Most common location?

Back 152

Polymyositis - most often involves shoulders

Front 153

What disease can cause present similarly to polymyositis (progressive symmetric proximal muscle weakness) with the addition of malar rash (similar to SLE), Gottron papules (on knuckles), heliotrope (erythematous periorbital) rash, "shawl and face" rash, and/or "mechanic's hands" appearance? What type of inflammation causes this presentation?

Back 153

Dermatomyositis: perimysial inflammation and atrophy with CD4+ T cells

Front 154

How does Polymyositis compare to Dermatomyositis in terms of location of symptoms?

Back 154

- Polymyositis: symmetric, progressive, proximal muscle weakness, especially in shoulders
- Dermatomyositis: same muscle weakness + malar rash, Gottron papules (knuckles), shawl and face rash

Front 155

What type of inflammation is responsible for Polymyositis and Dermatomyositis?

Back 155

- Polymyositis: endomysial inflammation with CD8+ T cells
- Dermatomyositis: perimysial inflammation and atrophy with CD4+ T cells

Front 156

What lab findings are associated with polymyositis and dermatomyositis?

Back 156

- ↑ CK
- (+) ANA Abs
- (+) anti-Jo-1 Abs
- (+) anti-SRP Abs
- (+) anti-Mi-2 Abs

Front 157

How do you treat a patient with polymyositis or dermatomyositis?

Back 157

Steroids

Front 158

What is the most common neuromuscular junction disorder?

Back 158

Myasthenia Gravis

Front 159

What is the pathophysiology responsible for Myasthenia Gravis?

Back 159

Auto-Abs to post-synaptic ACh receptor

Front 160

What are the symptoms of Myasthenia Gravis?

Back 160

- Ptosis
- Diplopia
- Weakness
- Worsens with muscle use

Front 161

What is Myasthenia Gravis associated with?

Back 161

- Thymoma
- Thymic hyperplasia

Front 162

How do you treat Myasthenia Gravis?

Back 162

AChE Inhibitor - reverses symptoms

Front 163

Which neuromuscular junction disease is more rare than Myasthenia Gravis?

Back 163

Lambert-Eaton Myasthenic Syndrome

Front 164

What is the pathophysiologic cause of Lambert-Eaton Myasthenic Syndrome?

Back 164

Auto-antibodies to presynaptic Ca2+ channels → ↓ ACh release

Front 165

What are the symptoms of Lambert-Eaton Myasthenic Syndrome?

Back 165

- Proximal muscle weakness
- Autonomic symptoms (dry mouth, impotence)
- Improves with muscle use

Front 166

What is Lambert-Eaton Myasthenic Syndrome associated with?

Back 166

Small cell lung cancer

Front 167

What is the effect of AChE inhibitors in patients with Lambert-Eaton Myasthenic Syndrome?

Back 167

Minimal effect

Front 168

What are the two neuromuscular junction diseases and how do they compare in terms of the cause?

Back 168

- Myasthenia Gravis: auto-Abs to post-synaptic ACh receptor
- Lambert-Eaton Myasthenic Syndrome: auto-Abs to presynaptic Ca2+ channel which decreases ACh release

Front 169

What are the two neuromuscular junction diseases and how do they compare in terms of the symptoms they cause?

Back 169

- Myasthenia Gravis: ptosis, diplopia, weakness, worsens with muscle use
- Lambert-Eaton Myasthenic Syndrome: proximal muscle weakness, autonomic symptoms (dry mouth, impotence), improves with muscle use

Front 170

What are the two neuromuscular junction diseases and how do they compare in terms of associated conditions?

Back 170

- Myasthenia Gravis: thymoma, thymic hyperplasia
- Lambert-Eaton Myasthenic Syndrome: small cell lung carcinoma

Front 171

If a patient has a suspicious "mass" at a site of trauma to their bone/muscle, what should you think of?

Back 171

Myositis Ossificans: metaplasia of skeletal muscle to bone (muscle becomes calcified) following muscular trauma

Front 172

Where is Myositis Ossificans more commonly found?

Back 172

Most often in upper or lower extremity

Front 173

What are the clinical findings in a patient with Myositis Ossificans?

Back 173

May present as a suspicious mass at a site of known trauma or as incidental finding on radiography

Front 174

What diagnosis should you consider in a patient with puffy and taut skin with an absence of wrinkles? Cause?

Back 174

Scleroderma (systemic sclerosis): excessive fibrosis and collagen deposition throughout the body

Front 175

Sclerosis can affect what organs?

Back 175

- Skin
- Renal
- Pulmonary (most common cause of death)
- Cardiovascular
- GI

Front 176

Who is more commonly affected by Scleroderma?

Back 176

Females (75%)

Front 177

What are the two types of Scleroderma (systemic sclerosis)?

Back 177

- Diffuse Scleroderma
- Limited Scleroderma

Front 178

What are the symptoms of diffuse scleroderma? Cause?

Back 178

- Widespread skin involvement, rapid progression
- Early visceral involvement (eg, renal, pulmonary, CV, GI)
- Associated with anti-Scl-70 Ab (anti-DNA topoisomerase I antibody)

Front 179

What are the symptoms of limited scleroderma? Cause?

Back 179

- Limited skin involvement confined to fingers and face
- Also with CREST involvement: Calcinosis, Raynaud phenomenon, Esophageal dysmotility, Sclerodactyly, and Telangiectasia
- More benign
- Associated with anti-Centromere Ab

Front 180

Which antibodies are associated with the two types of Scleroderma?

Back 180

- Diffuse Scleroderma: anti-DNA topoisomerase I antibody
- Limited Scleroderma: anti-Centromere antibody

Front 181

What are the symptoms of CREST involvement? What is it associated with?

Back 181

CREST:
- Calcinosis
- Raynaud phenomenon
- Esophageal dysmotility
- Sclerodactyly
- Telangiectasia

- Associated with Limited Scleroderma

Front 182

What type of lesion is this? Characteristics? Other examples?

Back 182

Macule
- Flat lesion with well-circumscribed change in skin color <1 cm
- Ex: freckles, labial macule (picture)

Front 183

What type of lesion is this? Characteristics? Other examples?

Back 183

Patch
- Macule that is >1 cm (flat lesion with well-circumscribed change in skin color)
- Ex: large birthmark (congenital nevus) - picture

Front 184

What type of lesion is this? Characteristics? Other examples?

Back 184

Papule
- Elevated solid skin lesion < 1cm
- Ex: mole (nevus) - picture, acne

Front 185

What type of lesion is this? Characteristics? Other examples?

Back 185

Plaque
- Papule >1 cm (elevated solid skin lesion)
- Ex: psoriasis (picture)

Front 186

What type of lesion is this? Characteristics? Other examples?

Back 186

Vesicle
- Small fluid containing blister <1 cm
- Ex: Chickenpox (varicella), Shingles (zoster)

Front 187

What type of lesion is this? Characteristics? Other examples?

Back 187

Bulla
- Large fluid-containing blister >1cm
- Ex: bullous pemphigoid

Front 188

What type of lesion is this? Characteristics? Other examples?

Back 188

Pustule
- Vesicle containing pus
- Ex: pustular psoriasis

Front 189

What type of lesion is this? Characteristics? Other examples?

Back 189

Wheal
- Transient smooth papule or plaque
- Ex: hives (urticaria)

Front 190

What type of lesion is this? Characteristics? Other examples?

Back 190

Scale
- Flaking off of stratum corneum
- Ex: eczema, psoriasis, SCC

Front 191

What type of lesion is this? Characteristics? Other examples?

Back 191

Crust
- Dry exudate
- Ex: impetigo

Front 192

What are the microscopic dermatologic term?

Back 192

- Hyperkeratosis
- Parakeratosis
- Spongiosis
- Acantholysis
- Acanthosis

Front 193

What lesion causes an increased thickness of the stratum corneum? Example?

Back 193

Hyperkeratosis - eg, psoriasis or calluses

Front 194

What lesion causes hyperkeratosis with retention of nuclei in the stratum corneum? Example?

Back 194

Parakeratosis - eg, psoriasis

Front 195

What lesion causes an epidermal accumulation of edematous fluid in the intercellular spaces? Example?

Back 195

Spongiosis - eg, eczematous dermatitis

Front 196

What lesion causes separation of epidermal cells? Example?

Back 196

Acantholysis - eg, pemphigus vulgaris

Front 197

What lesion causes epidermal hyperplasia (↑ spinosum)? Example?

Back 197

Acanthosis - eg, acanthosis nigricans

Front 198

What are the characteristics of hyperkeratosis? Examples?

Back 198

Increased thickness of the startum corneum
- Eg, psoriasis and calluses

Front 199

What are the characteristics of parakeratosis? Examples?

Back 199

Hyperkeratosis (↑ thickness of stratum corneum) with retention of nuclei in stratum corneum
- Eg, psoriasis

Front 200

What are the characteristics of spongiosis? Examples?

Back 200

Epidermal accumulation of edematous fluid in intercellular spaces
- Eg, eczematous dermatitis

Front 201

What are the characteristics of acantholysis? Examples?

Back 201

Separation of epidermal cells
- Eg, Pemphigus Vulgaris

Front 202

What are the characteristics of acanthosis? Examples?

Back 202

Epidermal hyperplasia (↑ spinosum)
- Eg, acanthosis nigricans

Front 203

What are the types of pigmented skin disorders?

Back 203

- Albinism
- Melasma (chloasma)
- Vitiligo

Front 204

What disorder has a normal melanocyte number with ↓ melanin production? Cause?

Back 204

Albinism
- Due to ↓ tyrosinase activity or defective tyrosine transport
- Can also be caused by failure of neural crest cell migration during development

Front 205

What causes Albinism?

Back 205

- Due to ↓ tyrosinase activity or defective tyrosine transport
- Can also be caused by failure of neural crest cell migration during development

Front 206

What is albinism associated with increased risk of?

Back 206

Skin cancer

Front 207

What disorder causes hyperpigmentation associated with pregnancy or OCP use?

Back 207

Melasma (Chloasma

Front 208

What disorder causes irregular areas of complete depigmentation? Cause?

Back 208

Vitiligo
- Caused by auto-immune destruction of melanocytes

Front 209

What are the common skin disorders?

Back 209

- Verrucae
- Melanocytic nevus
- Urticaria
- Ephelis
- Atopic dermatitis (eczema)
- Allergic contact dermatitis
- Psoriasis
- Seborrheic keratosis

Front 210

Which common skin disorder causes soft, tan-colored, cauliflower-like papules? Cause?

Back 210

Verrucae / warts - caused by HPV

Front 211

Which common skin disorder causes epidermal hyperplasia, hyperkeratosis, and koilocytosis?

Back 211

Verrucae / warts

Front 212

Which common skin disorder causes condyloma acuminatum on genitals? Cause?

Back 212

Verrucae / warts (caused by HPV)

Front 213

What common skin finding is this?

Back 213

Melanocytic Nevus - intradermal nevi are papular
- AKA common mole

Front 214

What common skin finding is this?

Back 214

Melanocytic Nevus - junctional nevi are flat macules

Front 215

What can melanocytic nevi lead to?

Back 215

They are benign, but melanoma can arise in congenital or atypical moles

Front 216

What common skin finding is this? Cause?

Back 216

Urticaria / hives - pruritic wheals form after mast cell degranulation

Front 217

How is urticaria characterized?

Back 217

Superficial dermal edema and lymphatic channel dilation, leading to pruritic wheals

Front 218

What common skin finding is this? Cause?

Back 218

Ephelis - freckles
- Normal number of melanocytes, with ↑ melanin pigment

Front 219

What common skin finding is this? Cause?

Back 219

Atopic Dermatitis (Eczema)
- Pruritic eruption, commonly on skin flexures

Front 220

What is the other name for Atopic Dermatitis? What is it associated with?

Back 220

Eczema
- Associated with other atopic diseases: asthma, allergic rhinitis

Front 221

What is the typical location of Atopic Dermatitis (Eczema)?

Back 221

- Usually starts on face in infancy (G)
- Often appears in antecubital fossae thereafter (H)

Front 222

What common skin finding is this? Cause?

Back 222

Atopic Dermatitis (Eczema)
- Pruritic eruption, commonly on skin flexures

Front 223

What common skin finding is this? Cause?

Back 223

Allergic Contact Dermatitis
- Type IV hypersensitivity reaction
- Follows exposure to allergen
- Lesions occur at site of contact (eg, NICKEL)

Front 224

What common skin finding is this? Cause?

Back 224

Allergic Contact Dermatitis
- Type IV hypersensitivity reaction
- Follows exposure to allergen
- Lesions occur at site of contact (eg, NEOMYCIN)

Front 225

What are common allergens that cause allergic contact dermatitis? What type of reaction?

Back 225

- Nickel
- Poison Ivy
- Neomycin

- Causes a type IV hypersensitivity reaction

Front 226

What common skin finding causes papules and plaques with silver scaling, especially on the knees and elbows?

Back 226

Psoriasis

Front 227

What common skin finding is this? What sign is this arrow pointing at?

Back 227

Psoriasis
- Auspitz sign - pinpoint bleeding spots from exposure of dermal papillae when scales are scraped off

Front 228

What are the skin findings in psoriasis?

Back 228

- Papules and plaques with silvery scaling (K), especially on knees and elbows
- Acanthosis (epidermal hyperplasia, ↑ spinosum) with parakeratotic scaling (nuclei still in stratum corneum)
- ↑ Stratum spinosum and ↓ stratum granulosum
- Auspitz sign: pinpoint bleeding spots from exposure of dermal papillae when scales are scraped off
- Can be associated with nail pitting and psoriatic arthritis

Front 229

What common skin finding appears as a flat, greasy, pigmented squamous epithelial proliferation with keratin-filled cysts and has an appearance of being "stuck on"? Cause? Where do these lesions occur?

Back 229

Seborrheic Keratosis
- Occurs on head, trunk, and extremities

Front 230

What common skin finding appears as a flat, greasy, pigmented squamous epithelial proliferation with keratin-filled cysts and has an appearance of being "stuck on"? Cause? Where do these lesions occur?

Back 230

Seborrheic Keratosis
- Occurs on head, trunk, and extremities

Front 231

What common skin finding appears as a flat, greasy, pigmented squamous epithelial proliferation with keratin-filled cysts and has an appearance of being "stuck on"? Cause? Where do these lesions occur?

Back 231

Seborrheic Keratosis
- Occurs on head, trunk, and extremities

Front 232

What is a common benign neoplasm of older persons?

Back 232

Seborrheic Keratosis

Front 233

What is the name of the sign for the sudden appearance of multiple seborrheic keratoses? What does this indicate?

Back 233

Leser-Trélat Sign: indicates an underlying malignancy (eg, GI, lymphoid)

Front 234

What are the types of infectious skin disorders?

Back 234

- Impetigo
- Cellulitis
- Necrotizing Fasciitis
- Staphylococcal Scalded Skin Syndrome
- Hairy Leukoplakia

Front 235

What skin disorder is characterized by "honey-colored crusting"? Most common causes?

Back 235

Impetigo
- Usually from S. aureus or S. pyogenes

Front 236

What kind of infection is Impetigo? Characteristics?

Back 236

- Very superficial skin infection
- Usually from S. aureus or S. pyogenes
- Highly contagious
- Honey-colored crusting

Front 237

What skin disorder is this? Most common causes?

Back 237

Bullous Impetigo - has bullae and is usually caused by S. aureus

Front 238

What skin disorder is this? Most common causes?

Back 238

Cellulitis
- Acute, painful, spreading infection of dermis and subcutaneous tissues
- Usually from S. pyogenes or S. aureus

Front 239

What often precipitates a Cellulitis infection?

Back 239

Often starts with a break in the skin from trauma or another infection

Front 240

What skin disorder causes bullae and a purple color to the skin? Most common causes?

Back 240

Necrotizing Fasciitis
- Deeper tissue injury, usually from anaerobic bacter or S. pyogenes
- Flesh eating bacteria

Front 241

What does Necrotizing Fasciitis lead to?

Back 241

Results in crepitus from methane and CO2 production

Front 242

What skin disorder causes fever and generalized erythematous rash with sloughing of the upper layers of the epidermis that heals completely? Cause?

Back 242

Staphylococcal Scalded Skin Syndrome
- Exotoxin destroys the keratinocyte attachments in the stratum GRANULOSUM only

Front 243

What happens if a patient is exposed to a staphylococcal exotoxin that can destroy keratinocyte attachments to the stratum granulosum?

Back 243

Staphylococcal Scalded Skin Syndrome
- Fever
- Generalized erythematous rash
- Sloughing of layers of epidermis
- Heals completely
- Seen in newborns and children

Front 244

What skin disorder causes white, painless plaques on the tongue that cannot be scraped off? Most common cause?

Back 244

Hairy Leukoplakia
- EBV mediated
- Occurs in HIV positive patients

Front 245

Who gets Hairy Leukoplakia? Appearance?

Back 245

- Seen in HIV-positive patients with EBV
- Causes a white, painless plaque on the tongue that cannot be scraped off

Front 246

What are the blistering skin disorders?

Back 246

- Pemphigus vulgaris
- Bullous pemphigoid
- Dermatitis herpetiformis
- Erythema multiforme
- Stevens-Johnson Syndrome

Front 247

What skin disorder is caused by IgG antibody against desmoglein (component of desmosomes)?

Back 247

Pemphigus Vulgaris

Front 248

What skin disorder is caused by IgG antibody against hemidesmosomes?

Back 248

Bullous Pemphigoid (think: antibodies are "BULLOW" the epidermis)

Front 249

Which skin disorder causes flaccid intraepidermal bullae and affects the oral mucosa? Cause?

Back 249

Pemphigus Vulgaris
- Auto-immune: IgG antibody against desmoglein (part of desmosomes)
- Caused by acantholysis (keratinocytes in stratum spinosum are connected by desmosomes)

Front 250

How can you diagnose Pemphigus Vulgaris?

Back 250

- Immunofluorescence reveals Abs (IgG against Desmoglein) against epidermal cells in a reticular (net-like) pattern
- Nikolsky sign (+) - separation of epidermis upon manual stroking of skin

Front 251

Which skin disorder causes tense blisters on the skin but spares the oral mucosa? Cause?

Back 251

Bullous Pemphigoid
- Auto-immune: IgG against hemidesmosomes

Front 252

How can you diagnose Bullous Pemphigoid?

Back 252

- Immunofluorescence reveals linear pattern at epidermal-dermal junction
- Tense blisters contain eosinophils
- Nikolsky sign (-) = separation of epidermis upon manual stroking of skin

Front 253

Which skin disorder causes pruritic papules, vesicles, and bullae (often on the elbows)? Cause / associations?

Back 253

Dermatitis Herpetiformis
- Deposits of IgA at the tips of the dermal papillae
- Associated with celiac disease

Front 254

Which skin disorder is associated with infections, drugs , cancers, and auto-immune diseases?

Back 254

Erythema Multiforme

Front 255

What infections can cause Erythema Multiforme?

Back 255

- Mycoplasma pneumoniae
- HSV

Front 256

What drugs can cause Erythema Multiforme?

Back 256

- Sulfa drugs
- β-lactams
- Phenytoin

Front 257

What type of lesions are associated with Erythema Multiforme?

Back 257

- Macules
- Papules
- Vesicles
- Target lesions (look like targets with multiple rings and a dusky center showing epithelial disruption)

Front 258

What skin disorder is characterized by fever, bulla, necrosis, sloughing of skin, and a high mortality rate?

Back 258

Stevens Johnson Syndrome

Front 259

Are mucus membranes affected in Stevens Johnson Syndrome?

Back 259

Yes - typically at least 2 mucus membranes are involved

Front 260

In what skin disorders ma you see target lesions?

Back 260

- Erythema Multiforme
- Stevens Johnson Syndrome

Front 261

What is the more severe form of Stevens Johnson Syndrome? How do you distinguish them?

Back 261

Toxic Epidermal Necrolysis
- >30% of the body surface area is affected

Front 262

Which skin disorder is associated with a hyperpigmented, velvety thickening of the skin? Cause?

Back 262

Acanthosis Nigricans
- Epidermal hyperplasia
- Associated with hyperinsulinemia (eg, diabetes, obesity, Cushing syndrome) and visceral malignancies (eg, gastric adenocarcinoma)

Front 263

What skin changes occur in Acanthosis Nigricans?

Back 263

Epidermal hyperplasia causes symmetrical, hyperpigmented, velvety thickening of skin, especially on neck or in axilla

Front 264

What premalignant lesion is caused by sun exposure, leading to a small, rough, erythamtous or brownish papule or plaque? What can it progress to?

Back 264

Actinic Keratosis: risk of squamous cell carcinoma is proportional to degree of epithelial dysplasia

Front 265

What is the appearance of the skin changes in Actinic Keratosis?

Back 265

Small, rough, erythematous or brownish papules or plaques

Front 266

What is there a risk of if you have Actinic Keratosis? What determines risk?

Back 266

Risk of squamous cell carcinoma is proportional to degree of epithelial dysplasia

Front 267

What is the skin disorder characterized by painful inflammatory lesions of the subcutaneous fat, usually on the anterior shins? Possible causes?

Back 267

Erythema Nodosum
- Often idiopathic
- Can be associated with sarcoidosis, coccidioidomycosis, histoplasmosis, TB, streptococcal infections, leprosy, and Crohn disease

Front 268

What is the appearance of Erythema Nodosum?

Back 268

Painful, inflammatory lesions of subcutaneous fat, usually on anterior shins

Front 269

What can cause Erythema Nodosum?

Back 269

- Often idiopathic
- Can be associated with sarcoidosis, coccidioidomycosis, histoplasmosis, TB, streptococcal infections, leprosy, and Crohn disease

Front 270

Which skin disorder is associated with the 6 P's: pruritic, purple, polygonal planar papules and plaques?

Back 270

Lichen Planus

Front 271

Which skin disorder manifests as Wickham striae (reticular white lines) on mucosal surfaces? What is it associated with?

Back 271

Lichen Planus
- Associated with Hepatitis C

Front 272

Which skin disorder is associated with a sawtooth infiltrate of lymphocytes at the dermal-epidermal junction?

Back 272

Lichen Planus

Front 273

What are the features of Lichen Planus?

Back 273

6 P's:
- Pruritic
- Purple
- Polygonal Planar Papules and Plaques

Mucosal involvement manifests as Wickham striae (reticular white lines)

Sawtooth infiltrate of lymphocytes at dermal-epidermal junction

Associated with Hepatitis C

Front 274

Which skin disorder is known as a "Herald patch" followed days later by a "Christmas tree" distribution of plaques with a collarette scale? How long does this last?

Back 274

Pityriasis Rosea
- Self-resolves in 6-8 weeks

Front 275

What is this finding? What is it a sign of?

Back 275

Herald Patch - sign of Pityriasis Rosea

Front 276

What is this finding? What is it a sign of?

Back 276

Christmas tree distribution - sign of Pityriasis Rosea

Front 277

What causes a sunburn?

Back 277

Acute cutaneous inflammatory reaction due to excessive UV irradiation

Front 278

What is the mechanism of damage causing sunburn?

Back 278

Causes DNA mutations, inducing apoptosis of keratinocytes

Front 279

What are the types of UV rays? How do they differ in the skin changes they cause?

Back 279

- UVA: causes tanning and photoaging
- UVB: causes sunburn

Front 280

What are the potential complications of sunburns?

Back 280

- Impetigo
- Skin cancers: basal cell carcinoma, squamous cell carcinoma, and melanoma

Front 281

What are the types of skin cancers?

Back 281

- Basal cell carcinoma
- Squamous cell carcinoma
- Melanoma

Front 282

What is the most common skin cancer?

Back 282

Basal Cell Carcinoma

Front 283

What kind of skin lesion is this? Characteristic appearance?

Back 283

Basal Cell Carcinoma
- Pink, pearly nodules
- Commonly with telangiectasias, rolled borders, and central crusting or ulceration

Front 284

What kind of skin lesion is this? Characteristic appearance?

Back 284

Basal Cell Carcinoma
- Non-healing ulcer with infiltrating growth

Front 285

What kind of skin lesion is this? Characteristic appearance?

Back 285

Basal Cell Carcinoma
- Scaling plaque (superficial BCC)

Front 286

What is the classic histologic appearance of a Basal Cell Carcinoma?

Back 286

- Palisading nuclei
- Nests of basaloid cells in dermis

Front 287

Where are most Basal Cell Carcinomas located?

Back 287

Found in sun-exposed areas of body

Front 288

What is the prognosis of Basal Cell Carcinoma?

Back 288

Locally invasive, but almost never metastasizes

Front 289

How can Basal Cell Carcinomas appear?

Back 289

- Pink pearly nodules, commonly with telangiectasis, rolled borders, and central crusting or ulceration
- Also can appear as non-healing ulcers with infiltrating growth
- Scaling plaque (superficial)

Front 290

What is the second most common skin cancer?

Back 290

Squamous Cell Carcinoma

Front 291

What is Squamous Cell Carcinoma associated with?

Back 291

- Excessive exposure to sunlight
- Immunosuppression
- Occasionally arsenic exposure

Front 292

What are the most common locations for Squamous Cell Carcinoma?

Back 292

- Face
- Lower lip
- Ears
- Hands

Front 293

What is the prognosis of Squamous Cell Carcinoma?

Back 293

Locally invasive, but may spread to lymph nodes and will rarely metastasize

Front 294

Which type of skin cancer causes ulcerative red lesions with frequent scales, and is associated with chronic draining sinuses?

Back 294

Squamous Cell Carcinoma

Front 295

What does Squamous Cell Carcinoma look like histologically?

Back 295

Keratin pearls

Front 296

What is the name of the scaly plaque, that is a precursor to Squamous Cell Carcinoma?

Back 296

Actinic Keratosis

Front 297

What is the name of the Squamous Cell Carcinoma variant that grows rapidly and may regress spontaneously? Timeline?

Back 297

Keratoacanthoma: grows rapidly over 4-6 weeks and regresses over months

Front 298

Which type of skin cancer has a significant risk for metastasis? What determine the risk of this?

Back 298

Melanoma - depth of tumor correlates with risk of metastasis

Front 299

What tumor marker is there for Melanoma?

Back 299

S-100 tumor marker

Front 300

What is Melanoma associated with?

Back 300

- Sunlight exposure
- Fair-skinned persons are at ↑ risk

Front 301

What are the key things to look for when evaluating a patient you think may have a Melanoma?

Back 301

ABCDEs:
- Asymmetry
- Border irregularity
- Color variation
- Diameter >6 mm
- Evolution over time

Front 302

What are the types of Melanoma?

Back 302

- Superficial spreading melanoma (I)
- Nodular melanoma (J)
- Lentigo maligna melanoma (K)
- Acrolentiginous melanoma (L)

Front 303

What skin lesion is this?

Back 303

Superficial Spreading Melanoma

Front 304

What skin lesion is this?

Back 304

Nodular Melanoma

Front 305

What skin lesion is this?

Back 305

Lentigo Maligna Melanoma

Front 306

What skin lesion is this?

Back 306

Acrolentiginous Melanoma

Front 307

What genetic abnormality often drives melanoma?

Back 307

BRAF kinase activating mutation (V600E)

Front 308

How do you treat Melanoma?

Back 308

- Primary treatment: excision with appropriately wide margins
- Metastatic or unresectable melanoma in patients with BRAF V600E mutation may benefit from Vemurafenib, a BRAF kinase inhibitor