AAW - Skin/MS Flashcards
supracondular fracture of the femur
what artery are you worried about
popliteal artery (runs behind the femur)
the superficial femoral artery becomes the popliteal after it runs through the adductor hiatus
CREST syndrome
and what autoantibody
Calcinosis, Raynaud phenomenon,
Esophageal dysmotility, Sclerodactyly, and
Telangiectasia.
a form of limited scleroderma
antiCentromere in Crest
pemphigus vulgaris mech of damage
characteristic “sign”
autoantibodies against desmoglein component of desmosome (macula adherens)
flaccid intraepidermal bullae caused by acantholysis (meaning the loss of intercellular connections) (keratinocytes in stratum spinosum are connected by desmosomes)
nikolsky sign - separation of epidermis upon manual stroking of skin
mech of bullous penphigoid
autoantibodies to hemidesmosomes (hemidesmosomes are down “bullow”)
spares the oral mucosa, and not as bad as pemphigus vulgaris
guyon canal syndrome
compression of the ulnar nerve at the wrist or hand, classically seen in cyclists due to pressure from handlebars
atrophy of intrinsic hand muscles, ischemia, pain, and edema due to vascular compression
what tumor may cause this
called thoracic outlet syndrome
caused by compression of lower trunk and subclavian artery
can be caused by pancoast tumor
you say “make a fist”
their 2nd and 3rd fingers stay straight
where is the lesion
you say “extend your fingers”
their 2nd and 3rd didgets stay flexed
where is the lesion
proximal median nerve - cannot flex
distal median nerve - cannot extend
same distal/medial dynamic goes for the ulnar nerve
Clawing is seen best with distal lesions because the remaining extrinsic flexors of the digits exaggerate the loss of the lumbricles - fingers are extended at the MCP, but flexed at the DIP and PIP joints
defects are less pronounced in proximal lesions; deficits present during voluntary flexion
injury to what nerve gives you foot drop
common peroneal
PED = Peroneal Everts and Dorsiflexes; if injured, foot dropPED
injury to what nerve presents with inability to invert and plantarflex the foot
tibial nerve
TIP = Tibial Inverts and Plantarflexes; if injured, can’t stand of TIPtoes
what molecule sits in the myosin binding groove on actin filaments and stops muscle contraction
what gets it out of the way
tropomyosin
Ca release binding to troponin C causes a conformation change that moves it out of the way
what bands in muscle shrink when contracted
H and I bands
(when you say HI to someone, you wave, which contracts your arm muscle)
A band is Always the same length
slow twitch muscle
1 slow red ox
type 1, red fibers are for slow, sustained contraction
denosumab
monoclonal antibody against RANKL
sometimes used to treat osteoporosis
think of a den full of bones
mutation that can be seen in osteopetrosis
carbonic anhydrase II
impaires ability of osteoclasts to generate acidic environment neccessary for bone resorption
patient has a lot of fractures, cranial nerve problems, pancytopenia
Dx?
Tx?
osteopetrosis
pancytopenia because bone fills marrow space
cranial nerve impingement and palsies as a result of narrowed foramina
Tx is bone marrow transplant (osteoclasts are derived form monocytes
patient says their hat size in increasing and they are having hearing loss, also have heart failure. serum Ca, phosphorus, and PTH are normal
disease?
histology?
serum finding?
paget disease (osteitis deformans)
localized disorder of bone remodeling caused by increase in both osteoblastic and osteoclastic activity
(hearilng loss from auditory foramen narrowing)
histo shows osteocytes within lacunae and chaotic, mosaic pattern of bone remodeling
get increased ALP
in osteitis fibrosa cystica, when do you get increased phophate? decreased phosphate?
increased - secondary hyperparathyroidism (e.g. ESRD where you cannot excrete PO4)
decreased - primary hyperparathyroidism
Li-fraumeni syndrome puts you at risk for what bone cancer
osteosarcoma
Li-fraumeni is a germline P53 mutation
genetics of Ewing sarcoma
patient age/sex
appearance of bone?
histo?
assc with 1(11;22) translocation
11+22 = 33 (patrick Ewing’s jersey number)
boys less than 15 years old (are what patrick ewing likes) (sorry patrick, this is my mneumonic)
onion skin appearance in bone (patrick ewing is as big as shrek, onions have layers thing was in shrek)
anaplastic small blue cells (the world is small and blue compared to a giant like patrick ewing)
expansile glistening mass within the medullary cavity
men 30-60 yo
what tumor
chondrosarcoma
soap bubble appearance of bone`
what condition
giant cell tumor
codman triangle on bone
indicative of osteosarcoma
vertebral fusion, stiff spine, uveitis, and aortic regurg
ankylosing spondylitis
adult male
dactylitis
asymmetric and patchy arthritis
they also have nail lesions and papules and plaques with silvery scaling on knees
psoriatic arthritis
HLA-B27
also see “pencil in a cup” on xray
someone had a GI illness then presents with
conjunctivitis
urethritis
arthritis
reactive arthritis (Reiter syndrome)
“can’t see, can’t pee, can’t bend my knee”
heart problems assc with lupus
kidney problems assc with lupus
libman-sacks endocarditis - wark like vegetations on both sides of valve. also you can find anticardiolipin antibodies (which can give a false positive on tests for syphilis)
lupus nephritis (type III hypersensitivity reaction) - either nephritis (diffuse proliferative glomerulonephritis) or nephrotic (membranous glomerulonephritis)
autoantibodies to postsynaptic ACh receptor = ?
antibodies to presynaptic Ca channels = ?
autoantibodies to postsynaptic ACh receptor = myasthenia gravis. give edrophonium to diagnose, get ptosis, diplopia, weakness that worsens with muscle use. assc with thymic hyperplasia
antibodies to presynaptic Ca channels = lambert-eaton myasthenic syndrome. Proximal muscle weakness, autonomic symptoms (dry mouth, impotence). improves with muscle use. (assc with small cell lung cancer)
widespread excessive fibrosis and collagen deposition throughout the body. sclerosis of skin, manifesting as puffy and taut skin.
rapid progression, early visceral involvement
what disease
what autoantibodies
diffuse scleroderma
anti-Scl-70 antibody (anti-DNA topoisomerase I antibody)
autoantibodies in CREST syndrome (limited scleroderma)
anti Centromere (C for CREST)
acantholysis
separation of epidermal cells
seen in pemphigus vulgaris
acanthosis
epidermal hyperplasia (increased spinosum)
seen in acanthosis nigricans
in what common skin condition to you see nuclei still in the stratum corneum
psoriasis
leser-trelat sign
sudden appearance of multiple seborrheic keratoses, indicating and underlying malignancy (GI, lymphoid)
celiac disease - what skin manifestiation
dermatitis herpetiformis
deposits of IgA at tips of dermal papillae, often found on elbows
things assc with acanthosis nigricans
hyperinsulinemia (diabetes, obesity, cushing)
visceral malignancy (gastric adenocarcinoma)
herald patch followed days later by “christmas tree” distribution
multiple plaques with collarette scale
what is it
tx?
pityriasis rosea
self resolving in 6-8 weeks
skin cancer that appears as a nonhealing ulcer or scaling plaque
what kind of cancer
nuclei?
basal cell
palisading nuclei
tumor marker for melanoma
mutation?
if they have the mutation, what drug can you give
s-100 (neural crest (mesodermal) origin) (neural crest keeps it 100)
also for neural tumors, schwannomas, langerhans cell histiocytosis.
BRAF kinase mutation (activating mutation) (zac braf has melanoma)
metastatic or unresectable melanoma in patients with BRAF V600E mutation may benefit from vemurafenib (melanomas look like a painting, or mural), a BRAF kinase inhibitor
LTB4
leukotriene B4
a neutrophil chemotactic agent. Neutrophils arrive B4 others
LTC4, D4, E4
do what
drugs that block them?
leukotrienes C4, D4, and E4
increase bronchial tone
zarfilukast, montelukast block the creation of them
PGI2
Prostaglandin I2
“Platelet-Gathering Inhibitor”
inhibits platelet aggregation and promotes vasodilation
allopurinol mech
drug interactions?
inhibits xanthine oxidase
increases concentrations of azathioprine and its toxic metabolite, 6-MP (normally metabolized by xanthine oxidase) - if you give both it can cause an aplastic anemia
febuxostat
inhibits xanthine oxidase
probenecid
inhibits reabsorption of uric acid in PCT (also inhibits secretion of penecillin)
used in gout
etanercept
fusion protein (receptor for TNF-alpha and IgG Fc) produced by recombinant DNA
etanerCEPT is a TNF decoy reCEPTor
infliximab
the DALI lama INFLIX pain on TNF-alpha
anti TNF-alpha monoclonal antibody
used in IBD, RA, ankylosing spondylitis, psoriasis
adalimumab
the DALI lama INFLIX pain on TNF-alpha
anti TNF-alpha monoclonal antibody
used in IBD, RA, ankylosing spondylitis, psoriasis
comon drug that causes gout
what do you see under a microscope of gout aspirate
thiazide diuretics
negatively birefringent crystals in joint aspirate*
15 month old
multiple skin nodules poor feeding pallor to conjunctivae multiple erythematous nodules on trunk raised tender spots on head oral ulcers
x-ray of head reveals circular lytic lesions
disease?
cells?
histo?
langerhans cell histiocytosis (histiocytosis X) - abnormal proliferation of langerhans cells, which are just are tissue-specific dendritic cells found only in the skin. they come from the bone marrow
dendritic cells, macorphages and b-lymphocytes make up the antigen-presenting cells (APCs), which are the only cells that express MHCII (which is used for CD4 t cell activation)
biopsy shows heterogenous mix of langerhans cells with eosinophils, neutrophils, histiocytes.
langerhans cells have tennis racket shaped vacuoles known as Birbeck granles
only muscle that comes from the third branchial arch
stylopharyngeus
used to elevate the pharynx and larynx
1st brancial arch derivatives
abnormalities?
chew (MMM)
maxillary process (maxilla, zygoMatic bone)
Mandibular process (Meckel cartilage, Mandible)
Malleus and incus, sphenoMandibular
Masseter, lateral and Medial pterygoids, Mylohyoid
pierre robin sequence is malformation of 1st and 2nd brancial arches - micrognathia, glossoptosis, cleft palate, airway obstruction
treacher collins syndrome - neural crest dysfunction –> mandibular hypoplasia, facial abnormalities
second branchial arch derivatives
Smile
reichert cartilage: Stapes, Styloid process, Stylohyoid
muscles of facial expression, Stapedius, stylohyoid, platySma
4th-6th branchial arch derivatives
ACCCT
arythenoids cricoid corniculate cuneiform thyroid
(used to sing and act)
4th: most pharyngeal constrictors; cricothyroid
6th: all intrinsic muscles of larynx except cricothyroid
branchial arches mneumonic
when at the restaurant of the golden arches, children tend to first chew 1, then smile 2, then swallow stylishly 3, or simply swallow 4, then speak 6
what makes up the spermatic cords:
internal spermatic fascia
cremasteric muscle and fascia
external spermatic fasci
ICE
tie
internal spermatic fascia - transversalis fascia
cremasteric muscle and fascia - internal oblique
external spermatic fascia - external oblique
bugs that cause palm and sole rashes
your drive CARS using your palms and soles
Coxsackie A, Rickettsia, secondary Syphilis
tick bite - rash starts centrally and spreads out, sparing palms and soles
what bug
what vecotr
typhus
endemic (fleas) - Rickettsia typhi
epidemic (human body louse) - Rickettsia prowazekii
rash - monocytes with morulae (mulberry-like inclusion) in cytoplasm
rash - granulocytes with morulae (mulberry-like inclusions) in cytoplasm
MEGA berry - a mulberry is a huge berry
Monocytes = Ehrlichiosis Granulocytes = Anaplasmosis
farmer has pneumonia and then develops endocarditis
not related to skin ms, but talked about with the other tick-borne rashes…
Q fever - Coxiella burnetii - can come from aerosols of cattle/sheep amniotic fluid
closely related to rickettsia but it is Queer because it has no rash or vector and its causative organism can survive outside in its endospore form
what arteries for anastomoses between the ulnar and radial arteries
the deep and superficial palmar arches
drugs that can cause stevens-johnson syndrome
some epilepsy drugs - ethosuxamide, phenytoin, fosphenytoin, carbamazepine, lamotrigine, allopurinol, sulfa drugs, penicillin
Steven johnson has epileptic allergy to sulfa drugs, allopurinol and penicillin
central american
multiple numb cutaneous erythematous skin plaques
lesions in extremities and buttocks
loss of eyebrows and lashes
epistaxis
what disease?
what cytokine can you give?
lepromatous leprosy
in lepromatous leprosy your Th cells are differentiated into Th2 instead of Th1. In individuals with Th1 responses, the macrophages respond to the interferon-gamma produced by the Th1 cells and you get “tuberculoid” leprosy.
can give interferon-gamma, which mimics the Th1 response
7yo that is now having trouble keeping up with other kids at recess. has hypertrophied calfs. normal reflexes, but decreased stregth
what disease
what inheritance
duchenne muscular dystrophy (x-linked recessive)
what is wrong in ehlers-danlos syndrome
differentiate between:
hypermobility type
classical type
vascular type
faulty collagen synthesis
hypermobility type - joint instability, most common
classical - joint and skin symptoms, mutation in type V collagen
(remember that type IV collagen defect is alport syndrome)
vascular - vascular and organ rupture, deficient type III collagen
drugs that cause drug induced lupus
HIPPES have lupus
Hydralazine, INH, Procainamide, Phenytoin, Etanercept, Sulfa drugs
14 yo
leg cramps after trying out for soccer
after tryouts, urine had reddish tinge
what disease
what is deficient
McArdle
glycogen phosphorylase in the muscle tissue, cannot cleave individual glucose-1-p molecules off of glycogen
24yo
sensorineural hearing loss, heart block, cerebellar ataxia, easy fatigability, proximal muscle weakness
myopathy diagnosed in childhood
muscle biopsy shows some red stuff in some cells
ragged red fiber disease
rare
mitochondrial inheritance
areas of unmineralized osteoid adjacent to normal trabeculae in a homeless man
disease?
labs?
osteomalacia
decreased vit d
decreased Ca
increased PTH
decreased phosphate
hyperactivity of osteoblasts leads to increased ALP
Rb gene is what
at risk for what cancers if it is mutated
gene product does what
tumor suppressor gene
RetinoBlastoma, osteosarcoma
gene product inhibits E2F, blocks G1–> s phase
tumor marker for hairy cell leukemia
Tx?
TRAP
Tartrate Resistant Acid Phosphate
The pure of heart, hairy man CLAD in armor is climbing the BINE stalk to TRAP the giant
causes marrow fibrosis - dry tap on aspiration
patients usually have massive splenomegaly
Tx - cladribine (purine analog, stops DNA synth) (a big, pure of heart hairy man clad in armor climbing the bine stalk), pentostatin
tick bite
arkansas
small, dark lesion
fever, malaise
gram neg coccobacillus, mostly intracellular
francisella tularensis
ticks, rabbits, deer flies
cellulitis and osteomyelitis from an animal bite
pasteurella multocida
nerve that innervates the adductor pollicis
ulnar (!) - also innervates the interosseus
the ulnar is on the medial side of the hand, think of the man with his palms showing anteriorly
genetic defect in achondroplasia
fibroblast growth factor receptor 3 gene
80% are spontaneous mutations, remaining cases are inherited in an autosomal dominant fasion
which muscle lowers the jaw while yawning
lateral lowers
m’s munch
lateral pterygoid opens
masseter, teMporalis, medial pterygoid munch
temporal arteritis is assc with what joint stuff
polymyalgia rheumatica
both are treated with low-dose corticosteroids
oral/facial abscess that drains through sinus tracts, forms yellow “sulfur granules;” can cause pelvic inflammatory disease with IUDs
causative agent?
Tx?
actinomyces
as with other gram positive bacterial with long, branching filaments resembling fungi, treatment is to SNAP the branches:
Sulfonamides for Norcardia
For Actinomyces, use Penicillin
drug used to treat osteoporosis that can cause stomach pain and hematemesis
-dronates
bisphosphonates
take them with a glass of water and remain upright for 30 min
what antibodies react to the cardiolipin antigen used in syphilis serology
what disease is it associated with
antiphospholipid antibodies
in lupus - also see anti-smith and ani-dsDNA
someone comes in with shoulder weakness and has anti-SRP antibodies
what is causing the damage
what disease
endomysial inflammation with CD8+ T cells (lymphocytes)
polymyositis - most often involves the shoulders
if there was a rash, dermatomyositis is on the DDx
what intrinsic muscle of the larynx is not innervated by the recurrent laryngeal nerve
the cricothyroid (derives from the fourth branchial arch)
whereas all of the others are derived from the 6th branchial arch
what is one rare kidney SE of a statin
rhabdomyolysis, leading to acute tubular necrosis
what things can cause malignant hyperthermia
succinylcholine and the inhaled anesthetics (-fluranes)
guy returns from vacation down south
exquisitely tender and enlarged lymph nodes. fever, chills, general weakness
painful ulcer with dark hemorrhagic purpura on the arm where a flea bit him
gets abnormal coagulation times
what organism
yersinia pestis (!)
achilles tendinitis
what antibiotic
fluoroquinolones (-floxacins)
umbilicated papules on the skin
what is it
molluscum contagiosum (poxvirus)
winged scapula
what is damaged - nerve, muscle, and spinal origin
occasional complication of what surgury
long thoracic nerve - branch of C5,6,7
innervates serratus anterior muscle
mastectomy
labs in paget disease
4 phases
increased ALP
normal phosphate and Ca
phases:
- osteoclastic
- mixed
- osteoblastic
- quiescent state - clastic and blastic activity is minimal
lesions in what area of the brachial plexus classically give you wrist drop and shoulder abduction
posterior cord - wrist drop is from radial nerve, shoulder abduction is from axillary nerve
rash starts in face and neck, moves to trunk and extremities
tounge initially had white coating but is now red.
red oropharynx
pale area around the mouth
what causes this
strep pyo exotoxin
this is scarlet fever
young patient uses his upper extremities to help himself get up
what type of mutation
frame-shift - x-linked recessive
this is the gower maneuver in deuschennes
difference between becker and duchenne
becker is a non-frameshift insertion in dystrophin gene.
onset is later and less severe
but they are both still x-linked recessive
looks like TB in an immunocompromised patient, but there is a negative PPD
what is it
what do you give to treat
Norcardia
aerobe
weakly acid fast
branching fillaments
SNAP the branches: sulfonamides for norcardia, actinomyces? use penicillin
type of bacteria that borrelia burgdorferi is
a corkscrew-shaped spirochete
good initial screening test for SLE because it is very sensitive
test to tell the prognosis of SLE
antinuclear antibody
it is not as specific as anti-dsDNA (bad prognosis) or anti smith
patient has a compression of the left recurrent laryngeal nerve
what muscle of the mouth is also innervated by the nerve that the recurrent laryngeal branches off ot?
the palatoglossus
vagus
muscle innervated by the glossopharyngeal
stylopharyngeus
someone is given an antibiotic for acne vulgaris
they they sunburn really bad
what is it/what is it’s mech of action
tetracycline
binds 30s ribosomal subunit
can also be used to treat rickettsial infections, cholera, lyme disease, and chlamydia and mycoplasma pneumoniae
patient has bad ring worm
what drug do you give
mech
SE - and why does it cause them (think endo hormones)
for bad tinea corporis, give ketoconazole
blocks formation of fungal membrane steroids
inhibits the enzymes desmolase, cyp 450, 17-alpha hydroxylase
inhibition of 17 alpha hydroxylase contributes to antiandrogenic effects like decreased libido, impotence, and gynecomastia
inhibition of desmolase results in skin darkening (desmolase is necessary for adrenal production of testosterone and cortisol)
with decrease cortisol, there is no feedback inhibition of the POMC, so you get addison like symptoms (hyperpigmentation)
ankle ligament that always tears first
ATF = always tears first = anterior talofibular
throwback: mech of sirolimus/rapamycin
for some reason, sirolimus sounded like a mountain of retreat to someone I know.
mTOR inhibitor, binds FKBP just like tacrolimus though
prevents response to IL-2
you can’t respond if you are on a mountain of retreat, also you don’t have to worry about you kidneys, but you might get panSIRtopenic
smooth and skeletal muscle:
which pathway uses troponin C?
which uses calmodulin?
which phosphorylates myosin?
muscle: troponin C, which alters the conformation of tropomyosin, allowing actin to bind directly to myosin
smooth muscle: calmodulin, which activates myosin light chain kinase, phosphorylating myosin directly, allowing it to for a cross bridge with actin
patient known to have an autoimmune disease undergoes a muscle stimulation test. the patient is initially unresponsive to the test, but after repetitive stimulation his muscle responds
lambert-eaton disease, antibodies against presynaptic voltage gated calcium channels
