Shared Flashcard Set

Details

Musculoskeletal and Connective Tissue
404-432 First Aid 2012 with errata corrections
141
Biology
Professional
01/18/2013

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Cards

Term
What are the 5 epidermal layers from surface to base?
Definition
Californians Like Girls in String Bikinis

1) stratum Corneum
2) stratum Lucidum
3) stratum Granulosum
4) stratum Spinosum
5) stratum Basalis
Term
What epidermal appendage performs each of the following functions?

1) Holocrine secretion of sebum from associated hair follicle

2) Sweat secretion throughout the body

3) Produces odorous milky, viscid secretion from axillae, genitalia and areolae, becoming functional after puberty
Definition
1) Sebaceous gland
- Holocrine secretions are produced in cytoplasm and released by membrane rupture and cell destruction

2) Eccrine gland

3) Apocrine gland
- odor is from bacterial action
Term
Describe the function of each of the following epithelial cell junctions.

1) Zona occludens
2) Zona adherens
3) Macula adherens
4) Gap junction
5) Integrin
6) Hemidesmosomes
Definition
1) Tight junctions are located near the apical faces of epithelial cells and prevent diffusion across paracellular space (Claudins and Occludins)

2) Intermediate junctions (just below zona occludens), where E-Cadherins connect to actin (CADherins are Calcium-dendent Adhesion molecules)

3) Also called Desmosomes, these are small, discrete sites of attachment, where cadherins connect to intermediate filaments composed of keratin and desmoplakin (Pemphigus vulgaris)

4) Allow adjacent cells to communicate for electrical and metabolic functions (found in between basolateral sides of adjacent epithelial cells)

5) Maintain integrity of basement membrane, binding laminin

6) Conenct cells to underlying ECM (bullous pemphigoid)
Term
What are the respective sites of autoantibody attack in pemphigus vulgaris and bullous pemphigoid, respectively?
Definition
1) Pemphigus vulgaris
- Macula adherens or "desmosomes," which are small, discrete attachment sites between adjacent epithelial cells, where cadherins attach to intermediate filaments like keratin and desmoplakin

2) Bullous Pemphigoid
- Hemidesmosomes, which connect cells and their BM to underlying ECM
Term
What are Cadherins and where are they found in the skin?
Definition
CADherins are Calcium-dependent ADhesion molecules

They are found at

1) Intermediate junctions (zona adherens), where they connect to intracellular actin

2) Macula adherens (Desmosomes), where they attach to intermediate filaments composed of keratin and desmoplakin.
Term
What might a positive "anterior drawer sign" indicate?

What about an abnormal "passive abduction" test of the knee
Definition
1) ACL (vs. posterior drawer for PCL)
- ACL is attached "anteriorly" to the tibia

2) MCL (vs. adduction for LCL)
- Lower limb is moved laterally away from body
Term
The posterior cruciate ligament attaches anteriorly to the tibia .
Definition
False: PCL means "posterior" attachement to Tibia.

ACL attaches to tibia anteriorly.
Term
Where should each of the following procedures be performed?

1) Pudendal nerve block

2) Locating Appendix at McBurney's point

3) Lumbar puncture
Definition
1) near Ischial spine during pregnancy

2) 2/3 of the way from ubilicus to anterior superior iliac spine

3) near Iliac crest
Term
What are the 4 muscles that form the rotator cuff, their function and their innervation?
Definition
SItS

1) Supraspinatous
- Abducts arm initially (before deltoid)- most common injury
- Suprascapular nerve (C5-6)

2) Infraspinatus
- Laterally rotates arm (pitching)
- Suprascapular nerve (C6)

3) Teres minor
- Adducts and laterally rotates arm
- Axillary nerve (C5)

4) Subscapularis
- Medially rotates and adducts arm
- Upper and lower subscapular nerve (C5-C6)
Term
What are the major bones of the hand?
Definition
Some Lovers Try Positions That They Can't Handle

Scaphoid, Lunate, Triquetrium, Pisiform, Trapezeum, Trapezoid, Capitate, Hammate.
Term
What is the most common bone injured in a fall on an outstretched hand?

1. Scaphoid
2. Lunate
3. Trapezoid
4. Triquetrium
5. Pisiform
Definition
1. Scaphoid
Term
What are the major hand bone divisions?
Definition
RA involves PIP and MP or CMC joints
Osteoarthritis involves PIP (Bouchard) and DIP (Heberden's) and

1) Carpal bones (wrist)

2) Metacarpal bones

3) Proximal phalanges

4) Distal phalanges
Term
What kind of nerve injury might each of the following scenarios produce?

1) Dislocation of humerus/fracture of cervical neck
2) Compressed cervical rib or Pancoast tumor or lung
3) Incorrect use of crutch
4) Supracondylar fracture of humerus
Definition
1) Axillary nerve (C5, C6)- Deltoid (shoulder abduction above 90)

2) Lower trunk of brachial plexus- Klumpke's palsy/claw hand

3) Radial nerve (C5-T1) compressed in axilla
- Saturday night palsy including loss of extension of the forearm, wrist, and proximal phalanges

4) Median nerve injury - Pronator teres syndrome
- pain and/or numbness in the distribution of the distal median nerve, and weakness of the muscles innervated by the anterior interosseous nerve
Term
What kind of nerve injury might each of the following cause?

1) Fracture of medial epicondyle of humerus
2) Subluxation of radius
3) Carpal tunnel syndrome
4) Dislocated lunate bone
5) Trauma to heel of hand and/or hamate fracture
Definition
1) Proximal ulnar nerve lesion (C8, T1)
- Medial 1 1/2 fingers and hypothenar eminence tingling
- Radial deviation of wrist upon flexion
- "Claw hand" on attempt to flex fingers

2) Radial nerve damage (C5-T1)
- Sensory loss on posterior arm and dorsal hand and thumb

3) Distal median nerve injury (C5-C8, T1)
- Dorsal and palmar aspects of lateral 3 1/2 fingers
- "Pope's blessing" on attempt to flex fingers
- "Ape hand" because you cannot oppose thumb

4) Also distal median nerve injury

5) Distal ulnar lesion (C8, T1)
- Lose abduction and adduction of fingers (interossei), Adduction of thumb and extension of 4th and 5th fingers (lumbricals)
Term
Consider the brachial plexus. Where might a lesion produce each of the following?

1) Waiter's tip, Erb's palsy
2) Claw hand, Klumpke's palsy
3) Wrist drop
4) Winged scapula
5) Deltoid paralysis
6) Saturday night palsy (wrist drop)
Definition
1) Upper Trunk lesion when C5 and C6 join
- loss of sensation in the arm and paralysis and atrophy of the deltoid, biceps, and brachialis muscles

2) Lower Trunk lesion (C8, T1)
- Claw hand with possible Horner's (T1) and ulnar sensory loss

3) Posterior chord

4) Long thoracic root (Serratus anterior) as it enters T1 root.

5) Axillary branches off of posterior chord (deltoid)

6) Radial branch injury off of posterior chord.
Term
Consider the brachial plexus. Where might a lesion produce each of the following?

1) Difficulty flexing elbow with variable sensory loss
2) Decreased thumb function, Pope's blessing
3) Claw hand and loss of intrinsic hand muscles
4) Deltoid paralysis
5) Winged scapula
Definition
1) Musculocutaneous branch off of lateral cord
2) Median branch
3) Ulnar branch
4) Axillary branch off posterior chord
5) Long thoracic
Term
Describe the anatomy of the brachial plexus
Definition
Randy Travis Drinks Cold Beer
Roots Trunks Divisions Chords Branches
1) Roots (C5-T1)

2) Trunks
- Upper (C5-C6)
- Middle (C7)
- Lower (C8-T1)

3) Divisions
- Each trunk gives off anterior and posterior divisions

4) Chords
- Posterior (three posterior divisions)
- Lateral (anterior divisions from the upper and middle trunks: C5-C7)
- Medial (anterior division of lower trunk: C8:T1)

5) Branches
- Musculocutaneous (lateral branch of lateral chord)
- Median (medial branches of lateral and medial chords)
- Ulnar (lateral branch of medial chord)
- Axillary (lateral branch of posterior chord)
- Radial (medial branch of posterior chord)
Term
What is the associated motor deficit with each of the following injuries?

1) Extended compression of axilla by back of chair or crutches

2) Fracture of supracondylar humerus

3) Fracture of medial epicondyle of humerus

4) Fracture of hook of hamate after falling onto outstretched hand.

5) Upper trunk compression

6) Dislocated lunate

7) Fracture of surgical neck of humerus
Definition
1) "Saturday night palsy" of Radial nerve (C5-T1)
- BEST extensors (Brachioradialis, Extensors of wrist and fingers, Supinator, Triceps)
- Wrist drop

2) Proximal median nerve lesion (C5-8, T1)
- Thumb opposition, lateral finger flex, wrist flexion
- Ape hand and "Pope's blessing"

3) Proximal ulnar nerve (C8, T1)
- Medial finger flexion and wrist flexion
- Ab/adduction of fingers (interossei)
- Radial deviation of wrist with flexion and claw hand

4) Distal ulnar lesion (C8, T1)
- Ab/adduction of fingers (interossei), extension of 4th and 5th fingers (lumbricals) and wrist flexion.
- Radial deviation of wrist with flexion and claw hand

5) Musculocutaneous (C5-C7)
- Biceps, brachialis, coracobrachialis
- Can't flex arm at elbow.

6) Distal median nerve injury
Term
What sensory deficit would be associated with each of the following signs?

1) Flattened deltoid
2) Wrist drop
3) Claw hand
4) Popes blessing hand
5) No flexion of arm at elbow.
Definition
1) Axillary nerve lesion (C5, C6)- no sensation over deltoid
- Problem with abduction of shoulder

2) Radial nerve injury (C5-T1)- No sensation over posterior arm and dorsal hand and dorsal thumb

3) Ulnar nerve injury (C8-T1)- No sensation over ventral 4th and 5th fingers and hypothenar eminence

4) Median nerve injury (C5-C8, T1)- No sensation over ventral later 3 1/2 fingers and thenar eminence)

5) Musculocutaneous (C5-C7)- No sensation over lateral forearm.
Term
Which muscle is NOT innervated by the Radial nerve?

1) Brachioradialis
2) Extensors of the wrist and fingers
3) Supinator
4) Coracobrachialsis
5) Triceps
Definition
Radial= BEST extensors
1) Brachioradialis
2) Extensors of wrist and fingers
3) Supinator
4) Triceps
Term
Why might a patient's arm look like a Waiter asking for a tip behind their back?
Definition
Erb-Duchene palsy: Tear of upper trunk (C5 and C6) following flow to shoulder or trauma during delivery.

1) Limb hangs by sides (abductor loss- deltoid)
2) Medially rotated (deltoid teres minor and infraspinatous)
3) Forearm pronation (loss of biceps)
Term
What is thoracic outlet syndrome and how does it relate to Klumpke's palsy?
Definition
TOS is embryonic or childbirth defect affecting inferior trunk of brachial plexus (C8, T1); cervical rib can compress the subclavian artery and inferior trunk, resulting in TOS.

1) Atrophy of thenar and hypothenar eminences (median and ulnar)

2) Atrophy of interosseous (finger abduction/adduction)

3) Sensory deficits on medial forearm and hand (not lateral, which is handled by musculocutaneous)

4) Disappearance of radial pulse upon moving the head toward the ipsilateral side

You get "Klumpke's palsy" Complete Claw appearance, because you lose lumbrical intrinsic muscles (flex MCP and extend IP), and forearm finger flexors (C5-7 median) and finger extensors (radial) are unopposed.
Term
True or False: A patient with a proximal median nerve lesion will present with "Pope's blessing" hand.
Definition
False.

With a DISTAL ULNAR Nerve lesion, the patient PRESENTS with "Pope's Blessing" hand.

With a PROXIMAL MEDIAN Nerve lesion, the patient must be asked to make a FIST before "Pope's Blessing" hand can be seen.
Term
A patient who recently underwent mastectomy presents with lymphoma and a winged scapula.

What happened?
Definition
Long thoracic nerve damage (C5-C7): Serratus anterior, which connects scapula to thoracic cage.

You also cannot abduct >50 degrees (you get half way with Deltoid)
Term
Describe the major muscles of the hand and their function.
Definition
Both groups perform same functions
Oppose, Abduct, and Flex (OAF)
DAB= Dorsal ABduct
PAD= Palmars ADduct

1) Thenar- Opponens pollicis, Abductor pollicis brevis, Flexor pollicis brevis
2) Hypothenar- Opponens digiti minimi, Abductor digitis minimi, Flexor digiti minimi
3) Dorsal interosseous (abduct)
4) Ventral interosseous (adduct)
5) Lumbrical- Flex MCP and extent PIP and DIP
Term
What motor defect is associated with each of the following injuries?

1) Anterior hip dislocation
2) Pelvic trauma
3) Knee trauma
4) Trauma to lateral aspect of leg or fibula neck
5) Posterior hip dislocation
6) Polio
Definition
1) Obturator (L2-L4)
- Thigh adduction
- Medial thigh sensory

2) Femoral (L2-L4)
- Thigh flexion and leg extension
- Anterior and medial leg sensory loss

3) Tibial (L4-S2)
- Foot inversion and plantarflexion; toe flexion
- Sole of foot sensory loss

4) Common peroneal (L4-S2)
- Foot eversion and dorsiflexion; toe extension; foot drop, foot slap, steppage gait
- Lose anterolateral leg and dorsal aspect of foot sense

5) Inferior gluteal (L5-S2)
- Can't jump, climb stairs or rise from seated position; can't push down

6) Superior gluteal (L4-S1)- could also be posterior hip damage
- Thigh abduction (Trendelenburg sign- contralateral hip drops when standing on leg ipsilateral to site of lesion)
Term
What motor deficit is associated with each of the following sensory defects?

1) Medial thigh
2) Sole of foot
3) Anterolateral leg and dorsal aspect of foot
4) Anterior thigh and medial leg
Definition
1) Obturator nerve (L2-L4), usually from anterior hip dislocation
- Thigh adduction

2) Tibial artery (L4-S2), usually from knee trauma
- foot inversion, plantar flexion of foot and dorsiflexion of toes

3) Common peroneal (L4-S2), commonly from trauma to lateral aspect of leg or fibular neck
- Foot eversion, dorsiflexion (see drop), toes extension (steppage gait).

4) Femoral nerve (L2-L4), commonly from pelvic fracture
- Thigh flexion and leg extension
Term
A patient is standing on his right leg and his left hip drops.

What is this sign and what is going on?
Definition
Trendelburg sign

Paralysis of hip abductor (gluteus medius and minimus) with damage to Superior Gluteal nerve (L4-S1), usually due to posterior hip dislocation or polio.
Term
True or False:

Damaged tibial nerve causes a foot drop.
Definition
False: The common peroneal does this

Peroneal (L4-S2) and Tibial (L4-S2) arise from Sciatic (L4-S2) in the posterior thigh.

PED= Peroneal Everts and Dorsiflexes; injured causes foot dropPED

TIP= Tibial Inverts and Plantarflexes; if injured, can't stand on TIPtoes
Term
Which of the following ligaments/menisci are NOT part of the "unhappy triad" football knee injury?

1) Medial collateral ligament
2) Anterior collateral ligament
3) Medial meniscus
4) Lateral meniscus
Definition
4) Lateral meniscus

Unhappy triad= MCL (abnormal passive abduction), ACL (anterior drawer) and MM
- Usually caused by force from lateral side
Term
Describe the basic molecular steps of muscle contraction. Which of the following is false?
Definition
1) Alpha motor neuron releases ACh, which binds post-synaptically and results in depolarization of motor end plate that travels along muscle cell and down the T-tubule.

2) Depolarization activates v-sensitive dyhydropyridine (L-type Ca 2+ channels) receptors in T-tubule membrane
, which causes coupled conformation changes in RxR on SR, leading to calcium release from SR into the cytosolic space.

3) Ca-binding to troponin C causes conformational change that moves tropomyosin out of myosin-binding groove on actin filaments.

4) Myosin releases bound ADP and Pi, and is displaced on actin filament (power stroke). Contraction results in H- and I-band shortening, but A band remains same length.

5) ATP binds myosin and allows it to release actin, and when ATP is hydrolyzed to ADP, Pi can again bind the the process begins again
Term
What is the cause of rigor mortis?
Definition
ATP depletion.

Myosin releases Pi and ADP during power stroke, but cannot release contraction without ATP.
Term
What is the difference between type 1 and type 2 muscle fibers?
Definition
One Slow Red Ox

1) Slow twitch; red fibers with many mitochondria and myoglobin (OX/Phos) enable sustained contractions

2) Fast twitch; white fibers due to fewer mitochondria and myoglobin (anaerobic glycolysis)
- These guys are hypertrophied by weight training.
Term
True or False:

The A band always remains the same length during shortening.
Definition
True!

A line Always the same (contains the entire length of a single thick filament: surrounds I band)

H shrinks (light zone of the thick myosin filaments that is not superimposed by the thin filaments)

I shrinks (region surround z line composed of actin)
Term
Describe the basic structure of the sarcomere
Definition
Sarcomere is segment between two neighbouring Z-lines, which are the discs that lie in between I bands.

Interaction between actin and myosin filaments in the A-band of the sarcomere is responsible for the muscle contraction, but A band is always the same length (H and I shrink)

- I (isotropic) band: the zone of thin filaments that surrounds Z line and is not superimposed by thick filaments.

- A (anisotropic) band surrounds I bands and contains the entire length of a single thick filament.

- H-band is the zone of the thick filaments within A band that is not superimposed by the thin filaments. Inside the H-zone is a thin M-line
Term
What is the role of each of the following proteins in skeletal muscle contraction?

1) Titin
2) Myomesin and C protein
Definition
1) Connects the Z line to the M line
- Contributes to force transmission at Z line and resting tension in surrounding I band (actin only band)

2) Found at M line, anchoring the thick filaments of the sarcomere (myosin) to other filaments, namely titin, stabilizing and aligning the structure
Term
What are the basic molecular events involved in smooth muscle contraction?
Definition
1) AP causes smooth muscle membrane (sarcolema) depolarization and calcium entry into cytoplasm

- Extra-cellar entry (L-type)
- Intracellular release from the SR (RxR)

2) Calcium binds calmodulin, which activates MLCK, phosphorylating myosin that is bound to actin

3) p-myosin + actin cause cross-bridge formation with contraction

**reversed by phosphatase**
Term
How does nitric oxide produce smooth muscle relaxation?
Definition
1) NOS produces NO, which activates GC and cGMP

2) cGMP activates PGK and phosphatase that inhibits MLCK

3) Without MLCK, myosin remains de-phosphorylated and will not induce cross-bridge formation with actin.
Term
What are the 2 basic processes of bone formation?
Definition
Osteoblasts arise from mesenchymal stem cells in periosteum.

1) Endochondral ossification
- Longitudinal growth where chondrocytes lay down cartilaginous model that is later replaced with woven bone by osteoclasts and osteoblasts
- Woven bone is remodeled to trabecular bone
**affected by FGFR3 mutations in achondroplasia**

2) Membranous ossification
- Flat growth (skull, facial bones and axial skeleton)
- Woven bone formed directly without cartilage precursor and then remodeled to lamellar bone
Term
True or False:

Membranous ossification involves flat bone growth of the skull, facial bones and axial skeleton where an initial cartilage model is laid down by chondrocytes and later replaced by woven bone by osteoblasts and osteoclasts.
Definition
False.

Membranous ossification IS flat growth of skull, facial bones and axial skeleton, but there NO INTERMEDIATE

Only endochondral ossification involves this intermediate, and produces longitudinal bone growth (affected in achondroplasia)
Term
Where are osteoblasts derived from?
Definition
Osteoblasts arise from mesenchymal stem cells in periosteum
Term
What autosomal dominant condition causes dwarfism with a normal life span and fertility?
Definition
Achondroplasia- Often sporadic and associated with older parents.

AD mutation in FGFR3 causing constitutive activation of the growth factor and inhibition of chondrocyte proliferation (Specifically affects endochondral ossification)

Kids have short limbs (endochondral), but normal/large head (membranous)
Term
What is osteoporosis and what are the 2 major forms?
Definition
1) Trabecular (spongy) bone loses mass and interconnections despite normal bone mineralization lab values.

Causes vertebral crush fractions- acute back pain, loss of height and kyphosis

2) Types
Type 1 (Post-menopausal): decreased estrogen prevents inactivation of IL-1, so osteoclasts go crazy and resorb bone.

- See Femoral neck fracture and distal radius (Colles' fractures)

Type 2 (Senile- men and women >70)
Term
What prophylactic strategies prevent the development of osteoporosis?

How can you treat it if it occurs?
Definition
Can be post-menopausal (type 1: low estrogen) or senile (type 2: >70).

1) Prevent with exercise and calcium ingestion before age 30

2) Treat with SERMS like tamoxifen or raloxifen (women) and/or calcitonin
- Bisphosphonates or pulsatile PTH for severe cases to prevent bone resorption

- NOT glucocorticodis.
Term
Which of the following strategies is NEVER appropriate for the treatment of osteoporosis?

1) Tamoxifen
2) Bisphosphonates
3) PTH
4) Glucocorticoids
5) Calcitonin
Definition
4!

SERMS and Calcitonin and regularly used, while PTH and Bisphosphonates (stick to calcium in bone and alter membranes of osteoclasts) are reserved for more severe cases.
Term
What are the complications associated with osteoporosis?
Definition
Vertebral crush fractures cause

1) Acute pain
2) Loss of height
3) Kyphosis
Term
Why do you see extra-medullary hematopoiesis in Osteopetrosis?
Definition
Marble bone disease, where genetic Carbonic anhydrase II deficiency in osteoclasts prevents H+ pumping and normal resorption (acidic environment is needed for dissociation of calcium hydroxyapatite from bone matrix)

Primary spongiosa fills medullary cavity an loss of marrow space causes anemia, thrombocytopenia and predisposes patients to infection.

With marrow loss, hematopoiesis is taken up by liver and spleen
Term
What laboratory values are associated with the disease that appears as "Erlenmeyer flask" bones that flare out of X ray?
Definition
Osteopetrosis- thickened, dense bones that are proned to fracture.

Carbonic anhydrase deficiency in osteoclasts prevents H+ pumping and normal resorption (acidic environment is needed for dissociation of calcium hydroxyapatite from bone matrix)

Serum calcium, phosphate and alkaline phosphatase (ALP) are NORMAL.
Term
Why might you see cranial nerve impingement in osteopetrosis?
Definition
Marble bone disease with deficiency in CA-II in osteoclasts (no H+ pumping) and lack of resorption.

Thickened bones produce narrowed foramina, which can impinge on exiting CN.
Term
How does Osteomalacia/rickets differ from Osteopetrosis?
Definition
Osteomalacia is "Soft bones" and Osteopetrosis is "Hard bones"

1) Osteomalacia is deficient bone mineralization (normal organic component) in adults with fused epiphyses (vs. kids, where it is rickets).

- Osteoid is soft, due to vitamin D deficiency (low vitamin D causes low calcium, which increases PTH secretion and decreased serum phosphate)

2) Osteopetrosis in abnormal osteoclast function (CA-II deficiency) and thick "marble" bones that are prone to fracture and cause CN impingement
Term
What is Osteitis deformans and why does it present with hearing loss?
Definition
Paget's disease (localized, vs. osteoporosis)

1) Abnormal bone architecture: increased osteoblast/osteoclast activity following viral infection (paramyxovirus)

- Serum calcium, phosphorus and PTH levels are normal, but ALP is increased (vs. Osteopetrosis, where ALP is normal)

- Can cause high output heart failure (increased BF through AV shunts) and osteogenic sarcoma

2) Auditory foramen narrowing.
Term
What are the classic X-ray findings of Paget's disease and what are the concerning outcomes?
Definition
Osteitis deformans (viral-mediated increase in osteoclast/osteoblast activity and localized architectural distortion)

1) Mosaic pattern; long bone chalk-stick fractures

2) Increased BF from AV shunts can cause high output HF
Predisposes to osteogenic sarcoma

3) Hearing loss if auditory foramen is impinged.
Term
What bone disorder is associated with each of the following collections of lab values?
(N)= normal (I)= increased (D)= decreased

1) sCa (N), Phosphate (N), ALP (N), PTH (N)- decreased mass

2) sCa (N), Phosphate (N), ALP (N), PTH (N)- thick, dense bones

3) sCa (D), Phosphate (D), ALP (I), PTH (I)

4) sCa (I), Phosphate (D), ALP (I), PTH (I)

5) sCa (N), Phosphate (N), ALP (I), PTH (N)
Definition
1) Osteoporosis (normal lab values)- spongey bone loss due to age or estrogen

2) Osteopetrosis (normal lab value)- CA-II deficiency with defective osteoclast H+ pumping and decreased resorption

3) Osteomalacia/rickets
- No vitamin D means no calcium. Increased PTH in response will decrease phosphate
- Elevated ALP means active bone formation

4) Osteitis fibrosa
- Hyperactive parathyroid leads to activation of osteoclasts and increased resorption
- Resorption increases sCa and increased PTH decreases Phosphate. Since bone is turning over, ALP is elevated

5) Paget's (Osteitis deformans)- viral disease causes increased osteoclast/osteoblast activity and disrupted architecture (high output HF, hearing loss and osteogenic sarcoma)
Term
Why might a patient with osteitis fibrosa cystica have hypophosphatemia?
Definition
Hyper-active parathyroids produce PTH, which increases serum Ca2+ and decreases phosphorous.

ALP would also be increased, because of bone turnover.
Term
What is Polyostotic fibrous dysplasia (PFD) and how does it relate to McCune-Albright syndrome?
Definition
1) PFD occurs when bone is replaced by fibroblasts, collagen and irregular bony trabeculae

2) McCune-Albright syndrome is a form of PFD with multiple unilateral bone lesions associated with endocrine abnormalities (precocious puberty) and cafe-au-lait spots.
Term
Which of the following descriptions does NOT fit a maligant primary bone tumor?

1) "Double bubble" or "soap bubble" appearance on X-ray

2) "Sunburst patter" on x-ray with Codman's triangle.

3) "Onion ring" appearance in bone associated with t (11;22)

4) Expansile glistening mass within medullary cavity
Definition
1) Giant cell tumors present this way (osteoclastoma) and are benign

1) Giant cell tumor (osteoclastoma)
- locally aggressive benign tumor of epiphyseal ends of long bones in people 20-40

- occurs around knee with spindle-shaped cells with multi-nucleated giant cells.

2) Osteosarcoma
- 2nd most common primary malignant bone tumor (after MM), occurring in the metaphysis of long bones (knee) in kids ages 10-20

- Codman's triangle is from elevation of periosteum (poor prognosis)

3) Ewing's sarcoma
- Extremely aggressive, anaplastic small blue cell tumor with metastasis, but responsive to chemo

- Boys <15 in diaphysis of long bones in the pelvis, scapula and ribs

4) Chondrosarcoma
- Malignant cartilaginous tumor (can be primary or arise from osteochondroma) in men 30-60

-Located in pelvis, spine, scapula, humerus, tibia or femur
Term
What kinds of bone tumors (benign and malignant) are found in each of the following bone regions?

1) Epiphysis
2) Metaphysis
3) Diaphysis
Definition
1) Epiphysis
- Giant cell tumors (osteoclastoma): benign (soap bubble)

2) Metaphysis
- Osteochondroma (exostosis): benign (cartilage cap)

- Osteosarcoma (osteogenic sarcoma): malignant (codmann's triangle and "Sunburst" on X-ray)

3) Diaphysis
- Chondrosarcoma: maligmant (expansive glistening mass of medullary cavity)

- Ewing's sarcoma: malignant ("onion ring" with 11;22 translocation)
Term
What types of primary bone tumors appear in each of the following age groups?

1) Men <25 years
2) Men 20-40
3) Men >40
Definition
1) <25
- Osteochondroma/exostosis (most common benign): cartilage cap in metaphysis

- Osteosarcoma (2nd most common malignant of bone): 10-20 years old with sunburst and codman's triangle in metaphysis

- Ewing's sarcoma (<15): diaphysis "onion skin" appearance with 11:22 translocation

2) 20-40
- Giant cell tumour (benign); epiphyseal "soap bubble" tumors with multi-nucleated giant cells)

3) >40
- Chondrosarcoma (30-60): diaphyseal glistening mass
(Primary or secondary to osteochondroma)
Term
What are the primary factors predisposing young boys to Osteosarcoma?
Definition
2nd most common primary bone tumor: occurring in metaphysis of long bones (often knee) in boys 10-20

1) Paget's disease of bone (viral architectural distortion)
2) Bone infarcts and/or radiation
3) Familial retinoblastoma

Look for cadman's triangle and "sunburst" on x-ray
Term
What benign primary bone tumor can rarely transform into chondrosarcomas?
Definition
Osteochondroma (exostosis): Cartilaginous caps in the metaphysis of young men (<25)

**most common benign tumor**

Chondrosarcomas a expansile glistening masses in the diaphysis of long bones in middle aged men (30-60)
Term
Patient presents with pain in his hips that gets worse as the day progresses and improves with rest. He is slightly bow-legged, but complains of no other systemic disturbances.

Which of the following is NOT associated with this condition?

1) Heberden's nodes in the DIP joints
2) Osteophytic bone spurs
3) Bouchard's nodes in the PIP joints
4) MCP inflammation
5) Eburnation
Definition
4- This is characteristic of RA, not osteoarthritis (which is described here).

OA occurs because of mechanical wear of joints that leads to destruction of articular cartilage, subchondral cysts and sclerosis.

Boney spurs, eburnation (polished, ivory-like appearance) and Heberden/Bouchard nodes are all classic
Term
Patient presents with pain in his hips that gets worse as the day progresses and improves with rest. He is slightly bow-legged, but complains of no other systemic disturbances.

Which of the following is NOT associated with this condition?

1) Heberden's nodes in the DIP joints
2) Osteophytic bone spurs
3) Bouchard's nodes in the PIP joints
4) MCP inflammation
5) Eburnation
Definition
4- This is characteristic of RA, not osteoarthritis (which is described here).

OA occurs because of mechanical wear of joints that leads to destruction of articular cartilage, subchondral cysts and sclerosis.

Boney spurs, eburnation (polished, ivory-like appearance) and Heberden/Bouchard nodes are all classic
Term
Why do patients with osteoarthitis often get bow-legged?
Definition
OA involves joint space narrowing, synovial hypertrophy, bone sclerosis and spurring, and loss of articular cartilage (wear and tear).

The medial meniscus is lost first, so patients are (usually transiently) bow-legged.
Term
What type of hypersensitivity reaction is associated with RA and how is it treated?
Definition
RA more common in females

1) Type III
- RF is an IgM autoantibody against IgG
- Anti-CCP antibody is less sensitive, but more specific

2) Treatment
- Symptoms: NSAIDS, COX-II inhibitors, TNF-ai, Glucocortiocoids
- Disease-Modifying (MTX, Sulfasalazine, hydroxychloroquine)
Term
What are the classic clinical features of RA?
Definition
Inflammatory arthritis affecting synovial joints, with pannus formation.

Symmetrical morning stiffness >30 minutes that improves with use, along with systemic involvement (fever, pleuritis, pericarditis)

1) MCP and PIP inflammation (NO DIP like OA)

2) Sub-q nodules (fibrinoid necrosis with palisading histiocytes)

3) + RF (sensitive) and anti-CCP (specific)

4) HLA-DR4 association
Term
What disease is associated with Boutonniere and Swan-neck deformities?
Definition
These are PIP deformities seen in RA, with ulnar deviation, rheumatoid nodules and MCP/PIP inflammation.

Treat with NSAIDS, COX-II inhibits, TNF-a inhibitors, steroids MTX, hydroxychloroquine, sulfasalazine
Term
What disease is associated with Xeropthalmia, Xerostomia and Arthritis.

What is the classic presentation?
Definition
Sjogrens (anti-SSa (Ro) and SSb (La)) with Parotid enlargement and risk of B-cell lymphoma and dental carries.

"I have dry eyes that feel like there is sand in them and I am having difficulty swalling"

- Xeropthlamia (dry eyes)
- Xerostomia (dry mouth and dysphagia)
- Arthritis (joint inflammation)

**Associated with RA and SICCA syndrome (dry eys, mouth, nasal cavity and vagina, with chronic bronchitis and reflux esophagitis, but no arthritis**
Term
What is SICCA syndrome and what condition is it associated with?
Definition
SICCA syndrome includes dry eyes, mouth, nasal cavity and vagina, with chronic bronchitis and reflux esophagitis, but no arthritis

Associated with Sjogren's (females 40-60), which has dry eyes, mouth and arthritis (parotid enlargement with ribonucleoprotein antibodies- Ro and La)

**Sjogrens is also associated with RA**
Term
What autoimmune condition presents with parotid enlargement and is associated with increased risk of B-cell lymphoma and dental caries?
Definition
Sjogren's (associated with SICCA and RA)

- Anti-SSa (Ro) and Anti-SSb (La)
- Dry eyes, mouth and arthritis
Term
How can you distinguish gout from pseudogout and how do you treat?
Definition
Tap for crystals

1) Gout
- Affects middle-aged men (90% under excretion)

- Monosodium urate (negative birefrigence-yellow, needle-shapped crystals under polarized light)

- Treat acutely with indomethicin or colchicine and chronically with Allopurinol (over-producer) or Probenecid (under-excreter)

2) Pseudogout
- Large joints like knee in older folks (male = female)

- Calcium pyrophosphate (positive birefrigence-blue, rhomboid-shapped crystals under polarized light)

- Treat acutely with NSAIDS and maybe steroids and colchicine
Term
True or False:

Calcium pyrophosphate crystals in pseudo-gout are blue when perpendicular to polarized light?
Definition
False! They are blue when PARALLEL,

Gout would be blue when perpendicular
Term
Which of the following is NOT a common cause of gout?

1) Lesch-Nyhan syndrome
2) PRPP excess
3) Thiazide diuretics
4) Calcium phosphate deposition
5) Tumor lysis syndrome
6) von Gierke's disease
Definition
4- This is PSEUDOgout

1) Buildup in uric acid because of deficiency in HGPRT enzyme

2) elevated purine breakdown and uric acid buildup
- seen in PRPP excess

3) Decreased excretion

5) Increased cell turnover

6) most common glycogen storage disease type 1
- glucose-6-phosphatase deficiency
Term
What are the classic acute/chronic symptoms of gout?
Definition
Asymmetric joint swelling with red, painful big toe (MTP is classic= Podagra)

1) Acute: large meat load (purines) or alcohol (enzymes compete for same excretion sites, so uric acid builds up)

- Treat with NSAID or Colchicine

2) Chronic cases produce Tophus (on external ear, olechranon bursa or achilles tendon)

- Treat with Allopurinol (over production) or Probenecid (under excretion)
Term
What are the most common causes of infectious arthritis?

1) Septic
2) Chronic
Definition
1) Septic
- S. aureus, Strep, N. gonorrhoeae are common

- Gonococcal arthritis presents with polyarticular, migratory arthritis with asymmetric distribution (STD= Synovitis/knee, Tenosynovitis/hand, Dermatits (pustules)

2) Chronic
- TB (disseminated)
- Lyme disease
Term
Sexually active male presents with recent-onset asymetric polyarticular migrating arthritis.

Affected joints include his left knee and MCPs. You also notice pustules all over his body.

What is most likely going on?
Definition
Septic arthritis- most likely Gonococcal (could be staph or strep as well)

If chronic, you would have to consider disseminated TB and Lymes disease.

STD= synovitis (knee), tenosynovitis (hand), dermatitis (pustule rash)
Term
What might cause hip pain with activity 2 months after trauma to that hip?

What other causes could lead to this same process?
Definition
Avascular necrosis (Osteonecrosis)

1) Trauma
2) High-dose corticosteroids
3) Alcocholism
4) Sickle cell
Term
What are the major forms of arthritis that occur without RF, but with a strong HLA-B27 association (HLA MHC 1) in males?
Definition
Seronegative Spondyloarthropathies= PAIR

1) Psoriatic arthritis (asymmetric and patchy)
- joint pain and stiffness with Dactylitis and "pencil in cup" deformity on X-ray
- seen in <1/3 of psoriasis patients.

2) Ankylosing spondylatitis (chronic)
- Ankylosis (stiff spine due to fusion of joints)
- Uveitis
- Aortic regurgitation

3) IBD-associated spondyloarthropathy (CD, UC)

4) Reactive arthritis (Reiter's)
- Urethritis, Conjunctivitis, Arthritis

Recall, Psoriasis, IBD and Chlamydia can also cause development of Ankylosing spondylitis in HLA-B27 + individual.
Term
Which patients can't see, can't pee and can't climb trees?

Which ones have "bamboo spines"
Definition
Seronegative spondyloarthropathies (HLA-B27 association)

1) Reactive arthritis (Reiter's)- Post-GI or chlamydia infections
- Conjunctivitis, Urethritis and Arthritis

2) Ankylosing spondylitis
- Chronic inflammation of spine with fusion (ankylosis)
- Uveitis and Aortic regurgitation also occurs.
Term
How can the acronym "I'M DAMN SHARP" help you make sense of SLE?
Definition
Immunoglobulins (anti-dsDNA, anti-Sm, anti-phospholipid)
Malar rash

Discoid rash
Antinuclear antibody
Mucositis (oropharyngeal ulcers)
Neurological disorders

Serositis (pleuritis, pericarditis)
Hematologic disorders (IgG warm autoimmune HA)
Arthtitis
Renal disorders (sub-endothelial deposits)
Photosensitivity
Term
Who gets SLE and when is it most severe?
Definition
90% are female (14-45)

Most common and severe is black females (especially with anti-dsDNA)

Fever, fatigue, weight loss, Libman-Sacks endocarditis (sterile, bilateral), hilar adenopathy and Raynaud's
Term
Which of the following lab test results is NOT associated with SLE?

1) False positive syphilis test (RPR/VDRL)
2) Positive ANA
3) Positive anti-dsDNA
4) Positive anti-SSa antibodies
5) Positive anti-histone antibodies
6) Positive Anti-smith antibodies
Definition
4- This is Sjogren's (dry eyes, mouth and arthritis associated with SICCA and RA)

- ANA is sensitive, but not specific

- anti-Smith and anti-dsDNA are both specific, but only dsDNA is prognostic (poor)

- anti-histone is drug-induced SLE

- false positive syphilis because of anti-cardiolipid antibodies that interact with cardiolipin in test.
Term
What disease is commonly associated with high serum ACE levels in black females and is associated with bilateral hilar lymphadenopathy?

What are the other classic signs/symptoms?
Definition
Sarcoidosis (Steroid treat)- Immune-mediated non-caseating granulomas that are widespread.

1) Restrictive lung disease (interstitial fibrosis)
2) Bilateral hilar lymphadenopathy
3) Erythemia nodosum
4) Bell's palsy
5) Epithelial granulomas with Schaumann and asteroid bodies
6) Hypercalcemia (elevated 1-alpha hydroxylase-mediated vitamin D activation in epithelioid macrophages)
7) Uveoparotitis
Term
Why do patients with Sarcoidosis get hypercalcemia?
Definition
Elevated 1-alpha hydroxylase-mediated vitamin D activation in epithelioid macrophages (non-caseating granuloma formation)

Treat with steroids
Term
What disease is associated with Shaumann and asteroid bodies within epithelioid granulomas?

How do you treat?
Definition
Sarcoidosis- Steroids

Black females with high ACE and restrictive lung disease!
Term
What disease is highly associated with older patients who are suffering from stiff, painful shoulders and hips, fever, malaise and weight loss, but with normal muscle strength?
Definition
Polymyalgia rheumtica is described- Giant cell arteritis

PR- Increased ESR but normal CK (not myopathy)
Treat with Prednisone
Term
Which of the following descriptions is NOT found in polymyalgia rheumatica?

1) Fever
2) Increased ESR
3) Muscle weakness
4) Normal CK
5) Temporal arteritis
6) Treated with prednisone
Definition
3- Muscle strength is normal (NOT a myopathy).

PR- shoulder/hip pain and stiffness with fever, malaise and weight loss in individuals >50.

Labs: Increased ESR and normal CK

Treat with prednisone
Term
Describe the typical presentation of Fibromyalgia.
Definition
Women (30-50) presents with widespread musculoskeletal pain associated with stiffness, paresthesia, poor sleep and fatigue.

MUST have >= 11 out of 18 tender points
Treat with NSAIDs and other pain management
Term
Which of the following is NOT characteristic of Polymyositis?

1) Progressive, symmetrical proximal muscle weakness
2) Gottron's papules on the DIPs
3) CD8+ T cell-mediated injury of myofibers
4) Increased CK and aldolase
5) Positive anti-Jo-1
Definition
2- This is characteristic of Dermatomyositis, which is similar to Polymyositis, EXCEPT for malar rash, Gottron's papules and Heliotripe rash (shawl on face)

Polymyositis is progressive symmetrical proximal (usually shoulders) muscle weakness caused by CD8+ T cell-inded injury to myofibers.

Labs show elevated CK, aldolase and positive ANA and anti-Jo-1

Treat withs steroids
Term
How are polymyositis and dermatomyositis treated? What are the characteristic laboratory findings?
Definition
Both involve progressive, symmetrical proximal muscle weakness (shoulders)

1) Labs
- Elevated CK and aldolase
- Positive ANA and anti-Jo-1

2) Treat with steroids
Term
Which of the following is NOT found in dermatomyositis?

1) Gottron's papules on DIP, PIP or MCP
2) Malar rash
3) Heliotrope rash
4) Discoid rash
5) Mechanics hands
6) Risk of malignancy
Definition
4- This is SLE

Dermatomyositis (positive ANA and anti-Jo-1, with CK and aldolase elevation) has Heliotrope and Malar rash, with papules and mechanics hands.

Watch out for malignancy as well
Term
What is the definitive diagnostic strategy for Dermatomyositis?
Definition
1) ANA and + anti-Jo-1 with elevated CK and aldolase suggest Polymyositis or Dermatomyositis

2) Muscle biopsy with perifascicular atrophy is diagnostic of Dermatomyositis.
Term
What is the difference between Myasthenia gravis and Lambert Eaton syndrome? How can you distinguish between the two diagnostically?
Definition
Autoimmune conditions affecting NMJ

1) Myasthenia gravis involves post-synaptic mACh receptors, causing ptosis, diplopia and general weakness

- Symptoms WORSEN with use (nerve stimulation/compound muscle AP test)
- Improvement with ACHE inhibitors (edrophonium test)

2) Lambert-Eaton syndrome involves pre-synaptic calcium channels, preventing ACh release and producing proximal muscle weakness.

- Symptoms IMPROVE with use
- No improvement with AChE inhibitor
Term
True or False:

Lambert-Eaton syndrome is associated with thymoma and improves with administration of AChE-i
Definition
False: This is Myasthenia Gravis (post-synaptic AChR at NMJ), which will worsen with use.

Lambert-Eaton (pre-synaptic calcium channels preventing ACh release) is associated with small-cell lung cancer and does NOT improve with these drugs. Will improve with use, however.
Term
Which autoimmune condition presents with ptosis, diplopia and general weakness in patients with h/x of thymoma?

Will their condition improve with muscle use?
Definition
Myasthenia gravis (attack of post-synaptic AChR).

They worsen with use and improve with AChE-i (opposite of Lambert Eaton, which is associated with small-cell lung cancer).
Term
What are the 2 major types of Scleroderma?
Definition
Excessive fibrosis and collagen deposition throughout body, commonly presenting with "puffy and taut" skin without wrinkles (75% female).

1) Diffuse- widespread skin involvement, rapid progression, early visceral involvement (anti-Scl-70/topo 1)

2) CREST- Calcinosis, Raynaud's, Esophageal dysmotility, Sclerodactyly, Telangiectasia

- Limited skin invovlment, often confined to fingers and face and more benign course.
- anti-Centromere antibody
Term
What autoimmune condition is associated with each of the following antibodies?

1) Anti-dsDNA
2) Anti-Scl70
3) Anti-SSa/SSb
4) Anti-centromere
5) Anti-histone
6) Anti-Jo-1
Definition
1) SLE (specific and prognostic-poor)
2) Diffuse Scleroderma (progressive systemic sclerosis)
3) Sjogren's syndrome (dry eyes, mouth and arthritis)
4) CREST syndrome (calcinosis, raynaud, esophageal, sclerodactyly, telangiectasia)
5) Drug-induced lupus
6) Polymyositis and Dermatomyositis
Term
Which has a poorer prognosis: Diffuse scleroderma or CREST syndrome?
Definition
Diffuse scleroderma (anti-SCl70/topo 1) has worse prognosis, with more rapid progression and early visceral involvement

CREST (anti-centromere) has limited skin involvement (fingers and face) and a more benign clinical course.
Term
What renal abnormalities are associated with SLE?
Definition
Major cause of death in SLE (other is infection)

Usually Nephritic > Nephrotic

1) Nephritic: Diffuse proliferative glomerulonephritis
- LM "wire looping of capillaries
- EM- sub-endothelial deposits and sometimes intramembranous IgG-based ICs with C3 deposition
- IF- granular "full house"

2) Nephrotic: Membranous glomerulonephritis (diffuse membranous glomerulopathy)

**can progress to DPGN**

- LM diffuse capillary and GBM thickening
- EM "spike and dome" appearance of sup-epithelial deposits
- IF- granular "full house"
Term
What kind of dermatological lesion is described by each of the following?

1) Flat discoloration <1cm
2) Flat discoloration > 1cm
3) Elevated skin lesion <1 cm
4) Elevated skin lesion > 1cm
5) Small fluid-containing blister
6) Transient papule or plaque
7) Large fluid-containing blister
8) Irregular, raised lesion from scar tissue hypertrophy
9) Blister containing pus
10) Dried exudates from vesicle, bulla or pustule.
Definition
1) Macule (Tinea versicolor)
2) Patch
3) Papule (Acne vulgaris)
4) Plaque (Psoriasis)
5) Vesicle (Chickenpox)
6) Wheal (Hives)
7) Bulla (Bullous pemphigoid)
8) Keloid (African american following trauma)
9) Pustule
10) Crust (Impetigo)
Term
What is the difference between a pustule and a vesicle?
Definition
1) Vesicle is small-fluid containing blister
2) Pustule is a blister containing pus, not fluid.
Term
What dermatological term is described by each of the following?

1) Increased thickness of stratum corneum
2) Increased thickness of stratum corneum with retention of nuclei
3) Separation of epidermal cells
4) Epidermal hyperplasia (increased spinosum)
5) Inflammation of skin
Definition
1) Hyperkeratosis (Psoriasis)
2) Parakeratosis (Psoriasis)
3) Acantholysis (Pemphigus vulgaris- desmosomes)
4) Acanthosis
5) Dermatitis
Term
What common skin disorder is described by each of the following?

1) Soft, tan-colored, cauliflower-like lesions with epidermal hyperplasia, hyperkeratosis, koilocytosis.

2) Common benign moles.

3) Intensely pruritic wheals that form after mast cell degranulation

4) Freckles (Normal number of melanocytes, with increased melanin pigment).
Definition
1) Verrucae (Warts)
- Condyloma accuminatum on genitals (HPV)

2) Nevocellular nevus

3) Urticaria (Hives)

4) Ephelis
Term
What is the difference between Atopic dermatitis and Allergic contact dermatitis?
Definition
1) Atopic (Eczema)
- Prurutic eruption on skin flexures in association with asthma and/or allergic rhinitis.

2) Allergic contact
- Type IV DTH reaction following exposure to allergen
- Lesions at contact site (nickel, poison ivey).
Term
True or False:

Allergic contact dermatitis involves type 1 hypersensitivity reaction.
Definition
False!

It is type IV, DTH, with lesions at site of contact (nickel or poison ivy)
Term
What disease is associated with papules and plaques with silvery scaling located on the knees and elbows?

What histopathalogical changes are characteristic?
Definition
Psoriasis

See Acanthosis with parakeratotic scaling (nuclei still in corneum)

Increased stratum spinosium and decreased granulosum (overlying layer)

Associated with psoriatic arthritis and nail pitting
Term
What is Auspitz sign?
Definition
Bleeding spots when Psoriatic scales are ripped off.

Remember, Psoriasis is associated with nail pitting and arthritis (Seronegative sponyloarthropathy with "pencil in a cut")
Term
Patient presents with sudden appearance of multiple flat, greasy, pigmented squamous epithelial proliferations with keratin-filled cysts on their head, trunk and extremities.

The lesions look like they are "pasted on horns."

Why is the doctor so concerned?
Definition
Sign of Leser-Trelat, with sudden onset of multiple "Seborrheic keratosis" with classic pasted on horn-like lesion.

**Make sure it is not melanoma**

Indicates underlying malignancy in elderly (GI, lymphoid)
Term
Describe each of the following pigmented skin disorders.

1) Albinism
2) Vitiligo
3) Melasma (cholasma)
Definition
1) Normal melanocyte number, but with decreased pigment due to decreased tyrosinase activity or failure of neural crest migration (opposite of freckle/Ephelis)

2) Irregular areas of complete depigmentation- decrease in melanocytes

3) Hyper-pigmented with pregnancy or OCP use
Term
What skin disorder is associated with each of the following?

1) Normal number of melanocytes with decreased production of melanin

2) Normal number of melanocytes with increased melanin production

3) Decreased number of melanocytes

4) Hyper-pigmentation during pregnancy or with OCP use
Definition
1) Albinism
- decreased tyrosinase activity or failure of neural crest migration

2) Ephelis (freckles)

3) Vitiligo (irregular areas of complete depigmentation)

4) Melasma (cholasma)- "mask of pregnancy"
Term
Which infectious skin disorder is described by each of the following? What etiologies are associated?

1) Highly contagious superficial skin infection with "Honey-colored crusting"

2) Acute, painful spreading infection of dermis and sub-Q tissues

3) Deep tissue injury with crepitus sounds from methane and CO2 production

4) Exotoxin-mediated destruction of keratinocyte attachments in stratum granulosum only, with fever and generalized erythematous rash and sloughing of upper epidermal layers.

5) White, painless plaques on tongue that cannot be scraped off
Definition
1) Impetigo (S. aureus or S. pyogenes)
2) Cellulitis (S. aureus or S. pyogenes)
3) Nec fasciitis (anaerobes and S. pyogenes)
4) Staph scalded skin syndrome (SSSS)
5) Hairy leukoplakia (EBV mediated in HIV (+) patients
Term
Patient presents with numerous crusted, denuded and wheapy erythematous plaques on her chest, breasts and arms.

When you stroke her skin, the epidermic separates with little effort.

What is the pathophysiology of this condition and how serious is it?
Definition
Lesions and Positive Nikolsky's sign indicates Pemphigus vulgaris

1) Autoimmune: IgG against desmosomes with "net-like" antibody pattern on IF of epidermis
- Acantholysis of skin and oral mucosa

2) Potentially fatal!
Term
Patient presents with multiple bullae on her arms and legs, but not in oral mucosa.

When you stroke her skin, the epidermic remains intact

What is the pathophysiology of this condition and how serious is it?
Definition
Bullous lesions sparing oral mucosa with negative Nikolsky's sign indicates Bullous pemphigoid.

1) IgG against hemidesmosomes (epidermal BM) with linear IF pattern
- Eosinophils in tense blisters

2) Less severe than pemphigus vulgaris
Term
Which of the following INCORRECTLY describes bullous pemphigoid?

1) Linear IF pattern of epidermal basement membrane
2) Negative Nikolsky sign
3) Eosinophils in tense blisters
4) Bullous lesions in skin and oral mucosa
5) Less severe than pemphigus vulgaris
Definition
4- Spares oral mucosa

All others are true (less severe than pemphigus vulgaris)

IgG against hemidesmosomes with eosinophils in tense blisters and negative Nikolsky sign.
Term
What are the major distinguishing features of Pemphigus vulgaris vs. Bullous pemphigoid?
Definition
1) IF
- Bullous is linear BM staining
- Vulgaris is reticular or "net-like pattern"

2) IgG target
- Bullous is hemidesmosome
- Vulgaris is desmosome

3) Nikolsky sign
- Bullous negiatve
- Vulgaris positive
Term
Describe the skin rash associated with Celiac disease
Definition
Dermatitis herpetiformis

- Pruritic papules and vesicles on elbows
- Associated with IgA deposits at tips of dermal papillae
Term
Which of the following is NOT a cause of Erythema multiforme?

1) Mycoplasma pneumoniae
2) Bactrim
3) Phenytoin
4) HSV
5) Strep pyogenes
6) Autoimmune disease
Definition
5- Group b strep is associated with impetigo and cellulitis, but not erythemia multiforme.

Multiple types of lesions (macules, papules, vesicles and target lesions (red papules with pale central area)
Term
What is "Stevens-Johnson" syndrome?
Definition
high mortality rate!

Fever, bulla formation and necrosis, and sloughing of skin from ADR

- If severe, called "Toxic Epidermal Necrolysis"
Term
Patient with hep C presents with skin rash. Describe it.
Definition
Lichen planus

- Pruritic, Purple, Polygonal Papules
- sawtooth infiltrate of lymphocytes at dermal-epidermal junction.
Term
Why are small, rough, erythematous or brownish papules forming "cutaneous horns" of clinical concern?
Definition
Actinic keratosis is premalignant (squamous cell) lesions caused by sun exposure. Risk of carcinoma is proportional to epithelial dysplasia.
Term
What is "Acanthosis nigricans"?
Definition
Hyperplasia of stratum spinosum, usually found in under-arms

- associated with hyper-insulinemia (Cushing's disease, diabetes) and Visceral malignancy
Term
What under-arm skin disorder is associated with Cushing's disease and visceral malignancy?
Definition
Acanthosis nigricans

- Hyper-insulinemia (Cushing and DM)
- Visceral malignancy
Term
Which of the following is NOT a common cause of Erythema nodosum?

1) TB
2) Mucormycosis
3) Histoplasmosis
4) Leprosy
5) Strep
6) Sarcoidosis
7) Coccidiomycois
Definition
Inflammatory lesion of sub-q fat on anterior shins

2: This is fungus with >90 degree angles
Term
What is the rash associated with a "herald patch" of papular eruptions, followed days later by a "Christmas tree" distribution that can remit spontaneously?
Definition
Pityriasis rosea

Benign, and usually (69%) following viral infection (HSV)
Term
Describe the common form of skin cancer that often develops from Actinic keratosis pre-curosor and rarely metastasizes.
Definition
Squamous cell carcinoma (sun or arsenic exposure)- usually on hands and face with keratin "pearls" on histopathology

- Ulcerative red lesion associated with chronic draining sinuses

- Keratoacanthoma is rapidly growing variant (4-6 weeks) with spontaneous regression (4-8 weeks)
Term
What is the rapidly growing variant (4-6 weeks) of squamous cell carcinoma that exhibits spontaneous regression (4-8 weeks)
Definition
Keratoacanthoma
Term
Patient from Miami pressed with a lesion on his nose that has rolled edges and an area of central ulceration.

You observe pearly papules and a telangiectasia

What do you tell him?
Definition
Basal cell carcinoma (sun-exposed area with characteristic lesion), which is a locally invasive, but non-metastatic tumor

- would see "palisading nuclei" on histo.
Term
Which of the following descriptions of basal cell carcinoma is INCORRECT?

1) Palisading nuclei on histopathology
2) Tends to metastasize
3) Lesion with rolled edges and central ulceration
4) Pearly papules
5) Telangiectasia
Definition
2- Locally invasive, but doesn't metastasize like Melanoma

Everything else fits (look in sun-exposed areas like nose)
Term
Which of the following regarding melanoma is FALSE?

1) S-100 tumor marker
2) High metastatic potential
3) Higher risk in fair skinned individuals
4) Actinic keratosis precursor
5) Dysplastic nevus precursor
6) Depth of tumor correlates with metastatic potential
Definition
4- This is SQUAMOUS cell carcinoma

For melanoma, look at ABCDs

Asymmetry, Border irregularity, Color variation, Diameter (>6cm) and history of change.
Term
What is the best prognostic maker of metastatic potential in melanoma?
Definition
Tumor depth

For diagnosis, look at ABCD (asymmetry, border, color, diameter)
Term
Describe the basics of the Arachidonic Acid pathway.
Definition
AA produces from Membrane lipid via Phospholipase A2

1) Lipoxygenase
- Hydroperoxides (HPETES) produce Leukotrienes
- LTB4 (PMN chemotaxis) and LTC4/LTD4 (increase bronchial tone)

2) Cyclooxygenase (COX-1 and COX-2)
- Endoperoxides (PGG2, PGH2) can become PGI2, PGE2, PGF2 or TXA2

- PGI2 (prostacyclin) decreases platelet aggregation, vascular tone, bronchial tone and uterine tone

- PGE2 increases uterine tone, decreases vascular tone and bronchial tone

- PGF2 and TXA2 increase platelet aggregation, vascular tone and bronchial tone.
Term
What is the mechanism of action of each of the following drugs?

1) Aspirin
2) Ibuprofen/Indomethacin
3) Celecoxib
4) Acetaminophen
5) Bisphosphonate (Etidronate, Pamidronate, ect.)
Definition
1) Irreversibly inhibits COX-1/2 by acetylation
- decrease synthesis of TXA2 and prostaglandins (increased bleeding time)
- bias toward Lipoxygenase pathway

2) Reversibly inhibit COX1/2 and block PGE/F synthesis

3) Reversible COX-2 inhibitor (spares gastric mucosa)

4) Tylenol: Reversibly COX inhibitor, mostly in CNS- inactivated peripherally.

5) Inhibit osteoclastic activity; reduce bone formation and resorption of hydroxyapatite
Term
What are the effects and toxicities of aspirin at low, intermediate and high doses?
Definition
1) Low (<300 mg/d)
- decrease platelet aggregation

2) Intermediate (300-2400 mg/d)
- antipyretic and analgesic

3) High (2400-4000 mg/d)
- anti-inflammatory

4) Toxicity
- Watch out for gastric ulceration, hyperventilation, tinnitus
- Chronic use (Interstitial nephritis and acute renal failure)
- Reye's syndrome in children with viral infection is a concern as well.
Term
Why might you use each of the following drugs and what are their important toxicities?

1) Celecoxib
2) Indomethacin
3) Acetaminophen
4) Pamidronate
Definition
1) COX-2 inhibitor used in RA and OA, or patients with gastritis or ulcers
- Risk of thrombosis, Sulfa allergy

2) NSAID used as anti-pyretic, analgesic, anti-inflammatory and to close PDA (inhibit PGE)

3) Anti-pyretic, analgesic (NOT anti-inflammatory) used instead of aspirin to prevent Reye's
- Hepatic necrosis (NAPQI)- give N-acetylcysteine to regenerate glutathione

4) Bisphosphonate- Osteoclast inhibitor used in malignancy-associated hypercalcemia (MM), Paget's disease of pone and post-menapausal osteoporosis.
- Corrosive esophagitis (except zoledronate), nausea, diarrhea, osteonecrosis of jaw.
Term
Why might you choose to prescribe Zoledronate (IV) instead of oral Pamidronate for your patient with post-menapausal osteoporosis?
Definition
Avoid corrosive esophagitis of other Bisphosphonates!
Term
Why might you give a child with a virus Acetominophen instead of Aspirin?
Definition
Avoid Reye's syndrome (kills the brain and liver!).
Term
What drugs are used for acute gout and what are their mechanisms?
Definition
1) NSAIDs (Naproxen or indomethacin)
- Reversibly inhibit COX1/2 for anti-inflammatory effect

2) Colchicine (not used much)
- Binds and stabilizes microtubules to inhibit polymerization , impairing leukocyte chemotaxis and degranulation

- Watch out for GI SE, especially when given orally.
Term
What drugs are used for chronic gout and what are their mechanisms?
Definition
1) Probenecid (under-excreter)
- Inhibits reabsorption of uric acid in PCT (also inhibits secretion of penicillin)


2) Allopurinol (over-producer)
- Inhibit Xanthine oxidase, which makes xanthine from hypoxanthine and uric acid from xanthine
- Used in Leukemia and lymphoma to prevent TLS_mediated urate nephropathy

3) Febuxostat (same as Allopurinol)
Term
What drugs should not be given with Allopurinol (or should be CLOSELY monitored)

What drugs should not be given period in a patient with chronic gout?
Definition
Remember, you give Allopurinol in Leukemia/lymphoma to prevent TSL-mediated urate neuropathy.

1) Azathioprine and 6-MP, because they are metabolized by Xanthine oxidase

2) Salicylates, which depress uric acid clearance.
Term
Describe the basics of purine metabolism and how it relates to use of Allopurinol.
Definition
1) Diet and Nucleic acids provide purines

2) Purines are metabolized to hypoxanthine, which becomes xanthine (Xanthine oxidase)

3) Xanthine becomes Uric acid (Xanthine oxidase) in plasma, which is filtered through kidneys.

Allopurinol inhibits Xanthine oxidase, so you get buildup of purines.

Probenecid prevents PCT reabsorption of uric acid, once it is filtered through glomerulus and enters the nephron.
Term
What are the major TNF-a inhibitors and how are they used clinically?
Definition
All are used in RA and Ankylosing spondylitis
Antibodies are used for CD
Etanercept is used in Psoriasis

1) Infliximab (anti-TNF antibody)
- CD, RA and Ankylosing spondylitis

2) Etanercept (recombinant human TNF receptor that binds TNF)
- RA, Psoriasis and Ankylosing spondylitis

3) Adalimumab (anti-TNF-antibody)
- RA, Psoriasis, Ankylosing spondylitis and CD
Term
What are the important side effects of TNF-a inhibitor use?
Definition
Infection

- reactivation TB because TNF blockage prevents activation of macrophages and destruction of phagocytosed microbes.
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