Skin/MS Pathology Flashcards
Trendelenberg sign
dropping of the lifted legs hip
Due to paralysis of the abductors of gluteus medius and gluteus minimus-> Superior gluteal invervation
Foot drop
Peroneal nerve damaged
dorsiflexes and everts the foot
PED
Can’t stand on tip toes
Tibial nerve damage
inverts and plantaflexes the foot
TIP
Thigh adduction innervated
- anterior hip dislocation
(4 muscles)
Obturator
adductor longu, adductor, brevis, anterior portion of adductus magnus, gracilis
Thigh flexion and leg extension nerve
Femoral
worry in pelvic fracture
Knee trauma worry about this nerve
Can
tibial
posterior hip dislocation and can’t jump
nerve and muscle
inferior gluteal enervating the gluteus maximus
pathology due to activating mutation in fibroblast growth factor receptor 3 (FGFR3)
Achrondroplasia - dwarfism
Achodroplasia
imparied cartilage proliferation in the growth plate
Dwarfism
Endochondrial bone formation
formation of bone via cartilage matrix which is replaced -> implicated in achondroplasia
Intramembranous bone fomration
formation of long bone w/out preexisting cartilage matrix - > flat bone formation (Skull and rib cage) thus why normal head in dwarfism
3 signs of osteogenesis imperfecta
multiple fractures (thinking child abuse)
Blue sclera
hearing loss
Thinning scleral collagen revealing choroidal veins
Defect in?
Blue sclera - in osteogenesis imperfecta
thing Autosomal defect in collagen type 1
Osteopetrosis is what and due to what?
inhereted defect in bone resorption -> abnormally thick and heavy bone
due to poor osteoclast activity - like carbonic anhydrase II mutations
Carbonic anydrase II mutatio nleads to
Osteopetrosis
can’t make acidic environment to resorb bone - osteoclasts do not work as well
Renal tubular acidosis w/ bone fractures is connected how?
lack of carbonicanhydrase II activity - osteopetrosis
- Normal enzyme activity leads to pumping of H in the lumen of tubules as NH4 and bicarb in the serum, lack of enzyme -> acidosis
myelopththisic process due to?
What causes?
Bone fractures
vision and hearing impairment
hydrocephalus
bone thickens out the marrow region leading o anemia, thrombocytopenia and leukopenia, as sen in osteoporosis
hearing and vision loss w/ nerve impingement
hydrocephalus w/ foramen magnum oclusion
Rx for psteopetrosis works how?
give bone marrow transplant so you have new source for monocyte derived osteoclasts
Defective mineralization of osteoid is called ?
Due to ?
rickets/ osteomalacia
due to VIt D deficiency, osteoblasts make osteoid but not mineralized
Active Vit D does what?
3 things
raises serum Ca and phosphate levels by acting on
intestine - increase absorb of Ca and phos
kidney - increase resorb of Ca and phos
bone - increase resorb of Ca and phos
1 alpha hydroxilation occur where after what>
in the proximal tubule of the kidney after 25 hydroxylation of Vit D
Frontal bossing, pigeon breast deformity, archaic rosary and what other symptom cause ?
What age group
Rickets in kids less than one
Usually also see bowed legs
Frontal bossing - large forehead
pigeon breast - inward bending of the ribs w/ anterior protrusion
rachitic rosary - ostoid deposition at chostochondral angle
low serum Ca and phos w/ increased PTH and alk phos seen in ?
Osteomalacia
Most common forms of osteoporosis (2)
senile - old age leads to loss of bone mass naturally w/out exercise or diet
postmenopause - lose of estrogen
Labs in osteoperosis
Serum Ca, phosphate, PTH and alk phos are NORMAL
Mosaic pattern on lamellar done is called?
Due to
Paget disease of the bone
Due to imbalance of osteoclast and osteoblast function in later 60s
Clinical features of pagets disease? (4)
Labs
bone pain - improper bone formation
hearing loss -impinged CN8
lion like facies
increasing hat size
- isolated alk phis levels due to isolated osteoblast activity post osteoclast activation (maybe viral)
Bisphosphates work in osteoporosis and Pagets due to
inhibition apoptosis of osteoclasts
Can also give calcitonin in pagets
2 complications in pagets
osteosarcoma - mutated cancerous osteoblasts
high output cardiac failure - AV shunts in bone -> high pressure
2 locations of osteomylitis in adults and kids
causes?
metaphysis in kids due to transient bacterium
epiphysis in adults due to open wounds
6 organisms implicated in osteomylitis
staph aureus - most common N gonorrhea - sexually active Salmonella - sickle cell pseudomonas - Diabetics and IV use pasteurella - Cat and dog bites mycopbaterium tuberculosis
Potts disease
TB and bone vertebrae implicated osteomyolitis
Cat and dog bites or scratches and you have osteomylitis caused by
pasteurella
Diabetics and IV drug use predispose this infectious organism for osteomyolitis
pseudomonas
Lytic focus of necrosis surrounded by sclerosis on x ray
clinical features?
osteomyolitis
Bone pain w/ fever and leukocytosis
Ischemic necrosis of the bone or marrow ->
Causes? (4)
avascular aseptic necrosis
Trauma
steroids
sickle cell anemia (dactelitis)
caisson disease - (gas emboli)
Gardner Syndrome (3)
Familial adenomys polyposis
fibromitosis in retroperitoneum
osteomas of the facial bone
bening tumor on the surface of facial bones
osteoma
young adult (<25) comes in w/ a tumor on the cortex of his long bone (the diaphysis)
x ray will reveal
osteoid osteoma
bony mass w/ radiolucent core - (osteiod)
Osteoblastoma differs from osteoid osteoma 3 ways
larger than 2cm
arises from the vertebrae - vs cortex of long bones
does not respond to ASA
Give ASA for bone pain w/ what tumor
osteoid osteoma
benign tumor of osteoblast
most common benign tumor of the bone grows where
Osteochdroma browns as a lateral projection of the growth plate - metaphysics
Osteochondroma concerns
transforms to chondosarcoma w/ overlying cartilage cap
Sunburst appearance and lifting of the periosteum -> cowman triage seen where?
Peak incidence?
in osteosarcoma
in teens and elderly
Osteosarcoma arises where?
Similar to
metaphysis of long bones - usually distal femur and proximal tibia
giant cell tumor except epiphysis
Risk factors for osteocarcoma (3)
familial retinoblastoma - teen
paget disease - elderly
radiation exposure - elderly
pleomorphic cells that produce osteoid seen on histology
osteosarcoma - malignant
Unique feature of giant cell tumor
only bone tumor arising in the epiphysis of long bone
- tumor comprised of multinucleated giant cells and stromal cells
Soap bubble appearance on x ray w/ a young adult
giant cell tumor
- locally aggressive and may recur
onion skin on x ray in a map child less than 15
ewing sarcoma
periosteum laying down new bone that is circumferentially compressed
malignant proliferation of poorly differentiated cells from neuroectoderm
Ewing sarcoma cells derived from
neuroectoderm
May present METS but highly treatable
small round blue cells that resemble lymphocyte differential in diaphysis of long bone (3)
Can have fever
Ewing sarcoma - 11:22 translocation
osteomylitis
lymphoma
11:22 translocation
Ewing sarcoma
Cartilage tumors usually arise from?
medulla of the of bone
small bones of the hand present w/ tumor
chondroma
tumor of medulla of the pelvis or central skeleton
chondrosarcoma
Most common bone tumor
See?
METS
See osteolytic punched out lesions
- EXCEPT prostate and breast cancer -> sclerosis and osteoblastic lesions (breast is also lytic)
Articular surface lining the synovial joint is made of what
What is secreted by synovium
hyaline cartilage type II
fluid rich in hyaluronic acid
Progressive degeneration of articular cartilage is what?
Affects which joints?
Osteoarthritis
DIP*, PIP, hips, lower lumbar spine, knees
Joint stillness in the morning that gradually gets worse throughout the day
Pathologically see (3)
Osteoarthritis
- joint mice- cartilage breaks into the synovium
- ebunartion or polishing of sub chondral bone
- osteophyte formation in the DIP
heberden nodes
DIP osteophite formation
Bouchard node
PIP osteophite formation
HLA DR4
Seen in rheumatoid arthritis
Synovitis leading to the formation of a pannus
Rhuematoid arthritis
inflammation -> grannulation tissue w/ associated fibroblasts, blood vessels and myfibroblasts (deviation and ankylosis)
See joint narrowing, loss of cartilage and osteropenia on X Ray
ankylosis
fusion - seen in rheumatoid arthritis
morning stiffness that improves w. activity
Rheumatoid arthritis
Break gown granulation tissue that build up over night throughout the day
Which joints are spared in RA
DIP
Symmetrical involvement otherwise
Clinical features of RA? (6)
Arthritis fever, malaise, weight loss Rheumatoid nodules Vasculitis bakers cyst Pleural effusions, LAD, intersitial lung fibrosis
Lab findings of RA
found in fluid
IgM autoantibody agianst Fc portion of IgG
Rheumatoid factor
See PMNs and high protein
Complications of RH(2)
Anemia due to chronic disease
secondary amyloidsosis, acute phase reactants SAA-> AA deposited
HLA B27
Seronegative spndyloarthritis
Seronegative Spondyloarthritis (3)
Meaning?
Ankylosing spondyloarthritis
Reiter syndrome
Psoriatic arthritis
Means lack of RA factor, HLA B27 and Axial skeleton involvement
Bamboo spine
Presentation and target group Extra articular (2)
ankylosing spondyloarthritis
young adults usually males w/ low back pain -> fusion of vertebra
Uveitis(red eye/blind)
aoritis (potential aneurism and regurgitation)
Reitor syndome qualified by (3)
Seen when
Urethritis, conjunctivitis, arthritis
-Cant see, can’t pee, can’t climb a tree
Post GI or chylamidia trachomatis infection
Sausage fingers/toes
Seen in sporadic arthritis - 10% of psoriasis
Axial and peripheral joints and DIP commonly
Infectious arthritis presentation and causal agents (2)
N gonorrhoeae - young adults
S aureus - older children and adults (2nd most common)
Single joint usually the knee
- warm joint w/ limited range of motion fever, leukocytosis and elevated ESR
Gout is due to generally -
hyperuricemia
Hyper purine metabolism or decreased excretion by the kidney
2 types of Gout (2; 1 and 3)
Primary - unknown cause due to hyper urea production and deposition of monosodium in tissues
Secondary
- Leukopenia and myeloproliferative disorders
- Lesch nyhan syndrome
- renal insufficiency
Deficiency in what enzyme leads to symptoms of mental retardation and self mutilation
Lesch Nyhan syndrome
- missing hypoxanthine guanine phosphoribosyltransferase
-> rescues hypoxthanthine and guanine before they are converted by xanthine oxidase xanthine and ultimately uric acid
Acute inflammatory reaction that is precipitated by consumption of alcohol or meats
Acute gout - commonly affects the big toe (podagra)
alcohol- slows excretion
meats - throws more purine into the system
tophi is made of what?
Associated w/
white chalky aggregates of uric acid crystals and fibrosis and giant cell reaction
- Chronic gout
Chronic gout leads to
renal failure w/ deposition of crystals in the kidney tubules
negative birefringence under polarized light
yellow, needle shaped, associated w/ hyperuicemia gout
rhomboid-shaped crystals w/ positive birefringence under polarized light
pseudogout
due to calcium pyrophosphate dihydrate deposition
malar rash w/ ANA and bilateral proximal muscle weakness
Dermatomysitis
NOT SLE
Clinical features of dermatomyositis (1 +3)
bilateral proximal muscle weakness-> distal weakness (can’t comb hair can’t climb stairs)
Rash on
- upper eyelid (heliotrope, purple)
- malar rash
- red papules on elbows, knuckles and knees
Anti jo 1 antibody
See also in lab (2)
dermatomyosisits
Also have ANA and increase creatine kinase
Perimysial inflammation (CD4 T cells) w/ perivascular atrophy
Dermatomyositis
- periphery of fascicle - closer to skin…(rashes)
differs from polymyositis w/ inflammation of endomysial inflammation w/ CD8
Polymyositis
-pathophys?
bilateral proximal muscle weakness w/out skin rash
- endomysial inflammation w/ necortic muscle fibers on biopsy
What needs to be ruled out with dermatomyositis?
underlying carcinoma especially gastric carcinoma
X linked muscular dystrophy(2) is due to what?
Duchenne -deletion of dystropin gene
Becker - mutation of dystropin gene
Dystophin is implicated in what and important for what?
in x linked muscular dystrophy
important in anchoring muscle cytoskeleton and EC matrix
Kid presents w/ calf that feels fatty around 1 yrs old
muscular dystrophy - X linked
Calf pseudohypertrophy
aslod find elevated serum creatine
distal muscles overworked when learning to walk and eventually replaced w/ fat (dystrophy)
cause of death in duchenne dystophy
due to replacement of myocardium or diaphragm w/ fat and have cardiac or respiratory failure
mutated dystophin leads to
beckers muscular dystophy, still have some protein made -> milder disease
presentation of muscle weakness that gets worse w/ use especially the eye (ptosis and diplopia)
pathophys?
Myasthenia gravis
Autoantobodies to the postsynaptic ACh receptor at the neuromuscular junction, less ACH w/ repeated use -> less ability to overcome the Ab blockade
Rx strategy in myasthenia gravis
acyetycholinesterase inhibitors -> more ACh around
Does not work in Lambert Eaton Syndrome due to lack of ini alt ACh w/ AB against the Ca channel
Association w/ myasthenia gravis and action?
thymic hyperplasia or thyoma
removal -> improvement in symptoms
Proximal muslce weakness that gets better w/ use and eyes are NOT affected
Patho-phys
Lambert Eaton Syndrome
Autoantibodies against the Ca channel presynaptically preventing depolarization and ACh release
Symptoms improve w/use due to increased Ca gradient -> eventual overwhelming of Ab
Lambert Eaton Syndrome etiology
due to paraneoplastice syndrome - often small cell carcinoma; resolves w/ removal
Lipoblast are characteristic of?
liposarcoma - most common malignant soft tissue tumor;
most common soft tissue tumor in adults benign
lipoma
Cardiac rhabdomyoma is associated w/ what syndrome
tuberous sclerosis
Rhabsomyoma - benign tumor of skeletal muscle
malignant skeletal muscle tumor?
Patient population and common site?
rhabdomyosarcoma
most common in kids
-vagina in girls; head and neck
rhabdomyoblast characteristic of ?
what is the name of the intermediate filament associated w/?
rhabdomyosarcoma
desmin
Layers of the skin top to bottom (4)
Stratum corneum
Stratum ganuosu,
Strum Spinosum
Stratum basalis
atopic dermatitis (eczema) location and description
flexor regions,
puritic erythematous oozing rash w/ vesicles and edema
Eczema is often associated w/
asthma and allergic rhinitis
Type 1 hypersensitivity
Contact dermatitis location and description
differs from eczema?
Where contact w/ allergen - poison ivy, nickel, drugs, irritants
puritic erythematous oozing rash w/ vesicles and edema
differs in type IV hypersensitivity
Comedome vs pustule vs nodule
comedome - white head/ black head blockage w/ excessive sebum and keratin production
Pustule/pimple is inflammation
nodule is scarred pustule
infectious agent in acne
MOA
proptionibacterium acnes
produces lipases that break down sebum releasing pro inflammatory FA
Rx for acne
benzoyl peroxide - antimicrobial
Vit A Isotretinoin - maintains specialized epithelial cells
Psorisis location and descripition
well circumscribed salmon colored plaque w/ silver scales
Extensor surface and scalp
may see pitting nails
HLA -C and areas of trauma
associated w/ triggering of psoriasis
Acanthosis
epidermal hyperplasia - psoriasis
parakeratosis
hyperkeratosis and retention of keratinocyte nuclei
-psoriasis
munro microabcesses
collection of PMNs in the stratum corneum
elongated dermal papillae the has thinned epidermis and picks when bleeds
auspitz sign
- psoriasis
5 Ps in Lichen Plansus
Puritic Planar Polygonal Purple Papule
Lichen Plansus normally attacks the (3)
wrists
elbos
oral mucosa - Wickham striae - reticular white lines)
Wickham striae -
reticular white lines of the oral mucosa in lichen planus
sawtooth appearance at the dermal epidermal junction
Associated w/ what infection
lichen planus
Chronic Hep C
autoimmune destruction of demosomes called?
occurs where?
pemphigus vulgaris
IgG attacks desmoglein (type II hypersensitivity) - separation of stratum spinosum
Skin AND oral mucosa
autoimmune destruction of hemidemosomses called?
Occurs where?
bullous pemphigoid
IgG attacks the hemidesmosome component of BM (BP180)
Only in the skin
Acantholysis
Separation of stratum spinsosum keratinocytes -> subrabasal blisters
pemphigus vulgaris
Nikolsky’s sign
Immunofloresence
thin walled bullae that easily rupture - does not include the BM w/ desmosome IgG attack
-> shallow erosions w/ dried crust
Seen in pemphigus vulgaris
Fish net w/ IgG attacking the desmosome,
Tense bullae that do not rupture, autoimmune disease of the skin
Immunoflorescene?
Bullous pemphigoid
Linear IgG Attacking the BM
Deposition of IgA at the tips of the dermal papillae?
Often due to ?
Dermatitis herpetiformis
- vesiculal lesions
Celiac
dematitis herpetiformis presentation (2)
puritic vesicles and bollae grouped - herpetiform
Targetoid rash and bullae that may be seen after a drug (penicillin) given is called?
erythema multiform
targetoid w/ central epidermal necrosis
Common cause of erythema multiform (5)
HSV - most comon Mycoplasma drugs (penicillin and sulfonamides) Autoimmune - SLE, Malignancy
Stevens Johns syndrome characterized by
Erythema multiform w/ oral mucosa/lip involvement and fever
diffuse sloughing go the skin resembling a large burn, often due to adverse drug reaction
Toxic epidermal necrosis
Progression of erythema multiforme -> steven johnson syndrome
Erythema nodosum is ?
acute, nodular, erythematous eruption that usually is limited to the extensor aspects of the lower legs.
- pain in subQ fat
most common form of panniculitis
Erythema nodosus cause (6)
tuberculosis, bacterial deep fungal infection, sarcoidosis, inflammatory bowel disease cancer
waxy stuck on appearance
See on histology
Seborrheic keratosis, common in elderly = extremities and face
see keratin pseudocyts on histology
Sudden onset of multiple seborrheic keratosis is called what? and worried of?
Leser Trelat sign
Worry about underlying carcinoma of GI
Acanthosis nigricans characterized by? (2)
Seen where? (2)
epidermal hyperplasia
darkening of the skin
-velvet like
axilla or groin
dark velvety skin seen on a patient may indicate 2 things
insulin resistance (DMII) malignancy - gastric carcinoma
Elevated nodule w/ cental ulcerates crater surrounded by dilated telangictatic vessel
pink pearly papules
Basal cell carcinoma
Common location and histology of basal cell carcinoma
upper lip(vs squamous cell)
basal cell nodules w/ peripheral palisading
Risk factors for skin carcinoma(3)
UVB induced DNA sunlight
Albinosim
Xeroderma pigmentosum - Auto recessive broken nucleotide excision repair mech
hyperkeratotic, scaly plaque on the face back or neck
Actinic kertatosis
precursor of squamous cell carcinoma
Ulcerated nodular mass on the face, maybe the lower lip
character feature
squamous cell carcinoma
keratin pearls
Additional risk factors for squamous cell carcinoma (3)
immunosuppressive therapy (most common CA post transplant)
arsenic exposure
chronic inflammation - burn or draining sinus
Keratoacnthoma
Presentation
well differentiated squamous cell carcinoma that develops rapidly and regresses spontaneously
Cup shape tumor filled w/ keratin debris
Melanocytes are derived from?
How do they pigment skin?
neural crest cell derived
synth melanin in melanosomes(tyrosine precursor) which are transferred to melanocytes
Vitilago pathophys
localized loss of skin pigmentation w/ autoimmune destruction of melanocytes
Albinism cause
Types(2)
enzyme defect (usually tyrosinase) impairing melanin production
Just ocular
both eyes and skin (oculocutaneous)
small tan - brown macule that darkens w. sunlight exposure due to?
freckle (ephelis)
increased # of melanosomes (NOT melanocytes)
Mask of hyper pigmentation w/ pregnancy or OCP
melasma
Congenital nevi can be differentiated by the presence of what
hair
Acquired nevi progression(3)
junctional nevus - nest of melanocytes at epidermal-dermal junction (kids)
compound nevus - extends to dermis
intradermal nevus - junctional component lost (adults)
Signs of melanoma
ABCD
Asymmetrical shape
irregular Border
irregular Color
Diameter >6mm
Addititional risk factor for melanoma in addition to sun
dysplastic nevus syndrome
Auto dominant disorder characterized by formation of dysplastic nevi
Most important facto in risk of METS in melanoma
depth into the dermis
Breslow thickness
1st radial growth horizontally
Lentigo maligna melanoma
lentoginous proliferation (radial) sub variant of melanoma
Similar to superficial spreading, another variant
Both good prognosis
Nodular melanoma
early vertical growth variant
poor prognosis
acral lentiginous
occurs where
w/ who?
melanoma arises on palms or soles
arises on dark skinned individuals w/out UV exposure
impetigo causes(2)
Presentation (3)
Stauph aureus and Streptococcal pyrogenes
*superficial
erythematous macules (flat)-> pustules on the face and eventual rupture -erosions and dry crusted honey colored lesions
Difference between impetigo and cellulitis
Bugs are the same
impetigo is superficial
cellulitis is deeper w/ red tender dwollen rash and fever
cellulitis risk factors(3)
recent surgery
trauma
insect bite
(indroducing S aureus and Streptococcal pyrogenes)
Surgical emergency surrounding cellulitis
associated feature?
necrotizing fasciitis w/ necrosis of sub Q tissue w/ anaerobic bacteria infection
Crepitus w/ production of CO2
Sloughing of skin w/ erythematous rash and fever due to ?
NOT just TENs
Also Staphylococcal scalded skin syndrome
-differs histiologically by separation at strum granulosum vs dermal epidermal junction
S aureus virulence factors causing dermicological dysfunction
Exfoliative A and B toxins -> epidermolysis of stratum ganulosum
Testicular METS gros to
peri-aortic lymph nodes
Ehlers Danlos syndrome is due to a defect in ?
Type V and sometimes type I collegen
Type III in vascular
Mutation leading to achondroplasia?
FGFR3
Achondroplasia is passed on how?
AD after a sporadic mutation in the germ line
-higher incidence w/ advanced paternal age
homozygous gene -> in utero fatality
FGFR3 mutation leads to what?
mutation characterized by?
Achrondroplasia
mutation leading to a constitutively active fibroblast growth factor receptor that inhibits chondrocyte proliferation so it won’t lay down new cartilage interfering w/ endochondral ossification
2 Benign bone primary bone tumors and associated age groups
Osteoclastoma seen in 20-40 yr olds
Osteochondroma seen in <25 yrs; males
2 Malignant primary bone tumors and associated age groups
Osteosarcoma seen in males 10-20
Ewing sarcoma seen in males <15
Soap supple appearance on x ray at the ditsal femur or proximal tibia
osteoclastoma (also called giant cell tumor)
-benign proliferation of osteoclasts
mature bone w/ cartilaginous cap seen on long metaphysics of bone
Osteochondroma
- benign
May be a type of haramtoma
Most common malignant bone tumor in kids
Osteosarcoma
2 X ray signs seen in osteosarcoma
Codmans triangle - periosteum is lifted off the bone by the tumor
sunburst sign - opacification
Oning skin appearance of the bone seen in what tumor and where
Translocation?
Ewing sarcoma - extremely aggressive
disruption and destruction of the diathesis of long bones, pelvis, scapula and ribs
T11:22
Common METS to the bone? (6)
Lytic vs blastic
More common than primary tumors
Permanently Relocated Tumors Like Bone
Prostate - Blastic Renal cell carcinoma Testes/ Thyroid Lung - Lytic Breast - BOTH
presentation of hypercalcemia and diffuse bone pain ?
think of METS to the bone
may have epidural spinal cord compression and associated sensory and motor deficits along w/ bowel dysfunction
Primary defect in osteoporosis?
Lab Values? Ca? Phos? ALP? PTH?
Overactive osteoclasts compared to osteoblasts
All normal
Primary defect in Pagets Disease of the bone?
Lab Values- Ca? Phos? ALP? PTH?
Overactive osteoclast and osteoblasts -> unorganized remedying of the bone (marbled)
Ca - normal
Phos - normal
ALP - increased due to turnover
PTH - normal
Primary defect in osteomalacia?
Lab Values? Ca? Phos? ALP? PTH?
Also known as rickets in kids, Vit D deficiency leading to inability of mineralize osteiod made by osteoblasts Ca- Low -> Increased PTH -> Low Phos -> increased ALP
Primary defect in osteopetrosis
Lab Values? Ca? Phos? ALP? PTH?
Mutation -> lack of carbonic anhydrase II leading to lack of osteoclast function
Increased ALP w/ thickened dense bone
may have decrease in Ca -> increase in PTH
Primary defect in osteitis fibrosa cystica
Lab Values? Ca? Phos? ALP? PTH?
Hyperparathyroidism either due to primary cause or due to type 1A psuedohyperparathyroidism (PTH resistance in renal tubules)
HIGH PTH
-> increased Ca
-> decrease in Phos
may or may not have increased ALP
Renal insuffiency will have what effect on bone density
Lab Values?
Lack of dihydroxylation of Vit D will lead to decreased Ca
- > increased PTH
- > INCREASED Phos (unable to excrete)
Brown tumors found where?
found in osteitis fibrous cystica where excess PTH due to primary hyperparathyroidism or psuedohyperathyroidsm (Renal receptors resistant to PTH)
-> excess osteoclastic activity and cystic lesions filled w/ fibrous stroma and blood found in bone
Bone replaced by fibroblasts, collagen and irregular trabecular is called?
Part of what triad?
polyostic fibrous dysplasia
Part of McCune -Albright syndrome
- precocious puberty
- cafe au lait spots
First line Rx w/ osteoperosis(3)
what also is considered?
bisphosphates
- alendronate
- risendronate
- etidronate
pulsitile PTH also
-Teriparatide
Behavior changes that prevent osteoporosis ((6)
stop
- smoking
- alcohol
- steroids
- H2 and PPIs (need H for Ca absorption)
- heparin (long term)
Weight bearing exercise
Concern w/ bisphophates (2)
erosive esophagitis
osteonecrosis of the jaw
Bisphosphates indicated for (3)
osteoporosis
Pagets disease of the bone
humoral hypercalcemia from malignancy
( PTH related peptide - paraneoplastic effect of small cell lung cancer for example)
bone pain, with bone enlargement and arthritis
Pagets disease of the bone
vertebral compression fractures
osteoperosis
Pagets disease of the bone is at an increased risk of what sequlae?
osteosarcoma
- may also have hearing loss w/ auditory foramen narrowing
Mutation in carbonic anyhdrase II is called?
Presentation? (3)
Osteopetrosis
Thick brittle bone-> fractures
cranial nerve impingement w/ excess bone
panyctopenia ( thrombocytopenia, infection) due to loss of bone marrow
RANK L inhibitor
Denosumab
-inhibits osteoclastic activity
-Remember osetoblast is the initiating factor that activates osteoclasts
SERM used for osteoporosis
Raloxifene
Tamoxifen used for Breast CA
Positive anterior drawer sign
ACL injury
Unhappy triad is due to what?
Composed of what?
lateral force applied to planted foot leading to damaged
ACL
MCL
meniscus (lateral more common than medial)
Housemade knee
prepatellar bursitis
clergyman knee
infraplatellar bursitis
Rotator cuff muscles and function
SItS
Supraspinatus - 1st 15 degrees of abduction
Infraspinatous - lateral rotation
teres minor - lateral rotation
Subscapularis - medial rotation
Most common torn rotator cuff muscle and test
Supraspinatous is most common torn rotator cuff muscle
Test with empty can test
Dislocated shoulder is
head of the humerus comes out of glenoid cavity
separated shoulder is
clavicle separates from the acromion process and coracoid process
subacromial buristis
impingement between head of the humorous and corocoaronial arch
shoulder impingement?
seen in?
acromion process compresses the supraspinatus tendon
repetitive overhead activities - baseball, tennis
Bones of the Wrist
So Long To Pinky Here Comes The Thumb
scaphoid - proximal lateral lunate triquetrum Pisiform - Proximal medial Hamate - distal medial Capitate Trapezoid Trapezium - distal lateral
pain in the anatomic snuff box break in
worry about?
scaphoid bone injury
necrosis to the proximal bone due to retrograde blood flow
Ankle sprain more likely to tear (3)
Lateral
- Anterior talofibular ligament
- Calcaneo fibular ligament
- Posterior talofibular ligament
Medial - rare
-deltoid ligament
Most hip locations occur where?
Risk of damaging what?(5)
posterior dislocation
medial circumflex and lateral circumflex of deep femoral artery
femoral vein,
femoral nerve
head of femur
Most common knee injury < 45?
Presents as?
Rx? (4)
Patellofemoral syndrome
anterior knee pain exacerbated w/ activity
strengthen quadriceps
stretching exercises - hamstrings, calves, hip, iliotibial band
minimize hard movement (jogging, stop and starts)
maximize walking and low impact
Pain beneath the calcaneus that is worse int he first few steps in the morning and after weight bearing?
Plantar fasciitis
Structures damaged by anterior shoulder location?
test what nerve by doing what?
axillary nerve
posterior circumflex artery
supraspinatous tendon
anterior glenohumeral ligaments- seperation
posteriolateral glenohumeral ligaments - abrasion
stroke the deltoid for sensation before reduction
Painless thickening of flexor tendons of the palms - usually affecting which finger?
Risk factors (4)
Dupuytrens contraction
- 4th
unknown etiology but risks:
- male
- > 40
- family
- alcohol/smoking
Frozen shoulder is?
adhesive capsulitis due to shoulder adhesions that lock the shoulder after disuse with an injury
especially if > 50 , woman and diabetic
Excessive bruising and bulge midhumoral think?
bicep tendon rupture
popye lesion
Large bump seen over the elbow that is painless
olecronon bursitis
Rx w/ needle to drain
tennis elbow is injury to?
lateral epicondylitis
inflamed lateral epicondyle and wrist extensors
golfers elbow?
medial epicondylitis
inflamed medial epicondyle and wrist flexors
eburnation
polished ivory like appearance of bone in OA
Radiographic changes in Osteoarthritis(3)
sclerotic changes
joint narrowing
ostrophyte formation
Presentation of Osteoarthritis
which joints in the hand(2)
Rx:
pain in weight bearing joints that gets worse during the day and with use
affects the DIP and PIP
-NO MCP
improves w/ rest, NSAIDs, acetaminophen (scheduled), hyaluronic acid, glucocorticoid injection, opiods
heberdens nodules vs bouchards nodules
osteophyte formation in OA in the DIP vs PIP respectfully
Predisposing factors for OA vs RA
age, weight and abnormal joint deformity vs
female and HLA DR4
Presentation of RA (4)
joints of the hands affected (2)
morning stiffness lasting >30 min,
gets better with use,
symmetrical joint involvement
w/ systemic features (fever, fatigue, pleuritis, pericarditis)
Affects the MCP and PIP
-NO DIP
Bakers cyst commonly seen w?
RA
Hypersensitivity type of RA?
Associated antibodies (2)
Type 3
Rheumatoid factor- IgM to IgG antibody (not specific)
Anticitrullinated peptide protein (ACPA) - more specific
Advanced RA of the hand pathology (4)
ulnar deviation of fingers
subluxation
Boutonnieres - flexed PIP and extended DIP
Swan neck deformity - extended PIP and flexed DIP
Rx for RA (5)
NSAIDs DMARDs - methotrexate -sulfasalazine -TNF alpha inhibitors -hydroxychloroquine
TNF alpha inhibitors (5)
Etanercept Infliximab adalimuxiab golimumab certolizumab
Radiographic findings in RA (3)
pannus formation
bone and cartilage erosion
increased synovial fluid
Uses of n acytylcystine?(3)
acetaminophen overdose
mucolytic in CF or intubation
nephropathy prevention w/ contrast
Risk of Celexicob use? (3)
thrombosis (not for those w/ CV family risk)
sulfa allergies
gastritis or ulcers (still some effect)
Renal problems w/ NSAIDS (2)
Others? (3)
Acute interstitial nephritis
Decreased renal perfusion w/ less prostaglandins keeping open the afferent arteriole
Gastritis/ peptic ulcer w/ COX1 inhib
fluid retention
aplastic anemia
how does Acetaminophen differ from NSAIDs?
Biggest toxicity concern?
peripherally inhibited
acts in the CNS as antipyretic and analgesic
NO anti inflammatory
The liver w/ NAPQI metabolites
ASA toxicity? (5)
gastric ulceration
tinnitus
acute renal failure
interstitial nephritis
hyperventilation -> respiratory alkalosis
-eventual lactic acidosis -> metabolic alkalosis
ASA effect on
bleeding time
PT/INR
PTT
Increases - anti platelet
No effect
No effect
Causes of Gout (3)
hyperuricemia -> deposition of monosodium irate crystals may be due to
increased purine food intake
decreased excretion - thiazide/loop diuretic use
Lesch Hyhan syndrome
increased cell turnover w/ Leukemia Rx
Podagra
painful swollen, red 1st MTP in gout
Gout acute attacks can be due to (2)
large meals
Alcohol use - competes for excretion w/ uric acid
Associated Gout findings(2)
Uric acid Tophi - external ear, olecranon bursa, achilles tendon
asymmetrical dystribution
Acute Gout attack drugs and MOA
NSAIDS- anti inflammatory
Colchicine - inhibits tublin -> decreased leukocyte chemotaxis
Steroids - anti inflammatory
Chronic Gout drugs and MOA (3)
Xanthine oxidase inhibitors
- allopurinol - not to be given acutely due to worsening/prolonging of symptoms
- febuxostat
- probenacid- inhibits uric acid reabsorption from Proximal renal tubule -> increased secretion
Chondrocalcinoisis
seen in pseudogout where Ca deposits in the articular cartilage (especially the meniscus of the knee) leading to Calcification seen on x ray
How does psuedogout differ from gout
Pseudo gout is rhomboid positive birefringent w/ parallel light
also usually affects large joints
Rx is the same
Pathology associated w/ HLA B27
PAIR - seronegative spondyloarthropathies
Psoriatic arthritis
ankylosing spondylitis
inflammatory bowel disease
Reactive arthritis
No Rheumatoid factor, more often males
Pencil in cup deformity seen on x ray?
psoriatic arthritis
especially the DIP
symptoms of psoriatic arthritis
Joints?
dactylitis
asymmetric and patchy involvement affecting the fingers (DIP), spine and sacroiliac
Uveitis and aortic regurgitation are often associated w?
How can you improve the primary problem
ankylosing spondylitis - bamboo spine
- can also have conduction abnormalities and CV disease
Exercise helps
Reactive arthritis is characterized by(3)?
Pathogenisis vs septic arthritis
Can’t see, can’t pee, can’t climb a tree
Conjunctivitis
Urethritis
Arthritis
Differs from septic in that it is a Rxn to the infection after the fact - fluid aspiration shows inflammation ~RA instead of pus and WBCs
Infections associated w/ Reactive arthritis (5)
Chlamydia
Post GI
-salmonella, Shigella, Yesernia, campylobacter, clostridium
3 forms of Juvenile Idiopathic arthritis
all have onset before 10
Polyarticular JIA(severe and symmetrical) Pauciarticular JIA (few large joints) systemic onset JIA (LAD, hepatomegaly, Rash, fever, elevated WBC, anemia prior to arthritis)
Infections causing septic arthritis?(3)
presentation (3)
Nessieria gonorrhea
S aureus
Streptococcus
polyarticular, migratory arthritis w/ asymmetric pattern
Associations w/ gonococcal arthritis (3)
Diagnose w?
SAD
synovitis - knee
tenosynovitis - hand
dermatitis - pustules
arthrocentesis
Check what before starting infliximab and the like?
PPD because TNF alpha inhibitors may reactivate latent TB
TNF alpha agents implicated for what arthritic processes(3)
RA
psoriases
ankylosing spondylitis
See elevated CK in this X linked muscle disease
Duchennes and Beckers
Difference between Duchene and beckers on molecular level
both X linked
Duchenne is a Deletion in dystophin -> accelerated mucle brakdown
beckers is a mutation
Qower’s manuever
seen in duchenne doe to pelvic muscles being weak and needing to use hands
Sjogren’s antibodies(3)
SS-A (rho)
SS-B (La)
occasionally rheumatoid factor or SLE
Triad of sjogrens syndrome
associated complications (3)
Xerophthalmia - dry mouth
Xerostomia - dry eyes
arthritis
parotid enlargment
B cell lymphoma risk
dental carries
ANA + in (7)
SLE sjogrens polymyostitis/dermatomystitis Scleroderma RA juvenile Idopathaic arthritis mixed connective tissue disease
Libman sacks endocarditis?
sterile vegitations seen in SLE
Common cause of death in SLE?
Check what to prevent?
lupes nephritis (wire loopis)
- Diffuse proliferative if nephritis
- membranous glomerulonephritis is nephrotic
Check serum creatinine and UA for proteinuria
Serum Antibody and changes associated w/ Lupus (5)
ANA Anti DNA - renal associated Anti Smith antiphospholipid (anti cardiolipin) - coag risk decreased C3 and C4 -infection risk
Drug induced lupus serum antibody
which drugs?
anti-histone
Sulfonamides Hydralazine INH Phenytoin Procainamide
What may be a false + in SLE
VDRL
Symptoms associated w/ SLE
-11 total
Skin(4)
- malar rash
- discoid rash
- photosensitivity
- painless oral ulcers
Inflam(3)
- arthritis (>2 joints, nonerrosive)
- serositis (pleuritis, percarditis)
- +ANA
Organ involvement(3)
- renal (proteinuria, casts)
- Neuro (seizure/psychosis)
- Heme ( anemia, low WBCs, platelets)
Immune(1)
- antiphospholipid, Anti DNA, Anti Smith, false VDRL
Polymyalgia rheumatica characterized by?
Associated w?
Labs?(2)
Differs from?
proximal joint pain
-shoulders and hips (Rx steriods)
Elevated ESR
Normal CK
Polymyosistis (proximal muscle pain)
Polymyalgia rheumatica associated w/ which vasculitis
temporal arteritis
proximal joint pain(shoulder and hip)
Anti jo antibodies
Polymyositis/dermatomyositis
difference between polymyositis and dermatomyositis(2)
Both have proximal muscle weakness, symmetrical
Skin involvement in dermatomyositis - malar gottrons papules on knuckles helitrope rash - eyes mechanics hands - rough hands and feet
CD4 in dermatomyocitis vs CD8
Skin involvement in dermatomyositis (4)
malar rash
gottrons papules on knuckles
helitrope rash - eyes
mechanics hands - rough hands and feet
Labs in polymyositis and dermatomyositis
CK?
Antibodies? (3)
elevated CK
ANA
Aldolase
anti-jo-1
Fibromyalgia is
associated signs?(6)
Rx(4)
Excessive muscle tenderness in 11 out of 18 trigger points
also - generalized pain, fatigue, sleep disturbance, HA, cognitive difficulties, mood changes
pregabalin - anti-convulsent
milnacipran -SNRI
Amytriptyline, fluoxitine
Proximal weakness vs distal weakness think
larger muscle disorders like polymyolitis vs distal think nerve disorders
anticentromere w?
CREST Scleroderma
anti toposiomerase w?
Also called
Scl-70
Diffuse scleroderma
Diffuse sceroderma vs CREST prognosis
Rapid widespread skin involvement, potentially fatal while Crest is limited in skin insolvent, often confined to fingers
CREST scleroderma stands for?
Calcinosis Raynauds Esophageal dysmotility Scleroderma Telangiectasia
Shiny skin w/ trouble swallowing ?
Check for in blood?
Scleroderma (CREST)
Anticentromere Ab
zona occuden uses what proteins (2)
tight junction - impermeable
claudin and occludin
zona adherins uses what proteins (2)
adhering junctions
belt w/ actin filaments
and CADherins - Ca dependent adhesion proteins
macula adhering uses what proteins
desmosomes to connect cells
keratin interactions w. cadherins
-pemphigous vulgaris
Gap junction connects between cells using what protein
connexons
Hemidesmosomes use what proteins
connects to basement membrane w/ keratin
integrins also
bullous pemphigoid
melanocytes are found in the
stratum basale
Fibroblasts are important in producing(4)
collagen
glycosaminoglycan
reticular and elastic cells
glycoprotein
The itch that rashes
Rx: (6)
atopic dermatitis
moisturizers w/ emollient calcineuron inhibitors (tacrolimus, primecromlimus) UV light - better in the summer topical steriods antihistamine leukotriene inhibitors
Common location of atopic dermatitis
in the flexors - behind the knee and in the elbow
Maybe all over a baby that is being cleaned too much
verrucae
wart
ephelis
freckle
excess melanin NOT melanicytes
nevi
mole
Linear puritic rash
what type of hypersensitivity
contact dermatitis - poison ivy or nickel
Type IV
4 causes and associated Rx w/ Acne
hyperkeratosis - retinoids, isotretinion
sebum overproduction - isotretinion, OCPs, spiranolactone
propioni bacterium acne - erythromycin, tetracyclin, doxy, minocyclycline, clindamycin, benzoyl peroxide
inflammation - steriods
isotretinion, OCPs, spiranolactone are all used in RX for
acne
Skin disorder associated w/ asthma and allergic rhinits
atopic dermatitis
eczema
keratin filled cysts - horn cells
seberrheic keratosis
increased stratum spinosum w/ decreased stratum granulosum w/ parakeratotic scaling?
psoriasis
Psoriasis is often found where?
on the extensor surface (NOT in folds)
knees and elbows
depigmentation due to decreases melanin production
cause?
ambinoism
decreased tyrosinase activity
depigmentation due to decrease in menanocyte #
cause?
vitiligo
??
darkening of the dace seen in pregnancy or OCP
melasma
flaccid blisters w/ Nikolskys sign?
Involve the mouth?
phemphigus vulgaris - IgG against desmoglein 3 or 1
skin just sloughs off
involves the mouth
tense blisters w/out Nikolskys Sign?
Involves the mouth
Bullous pemphigoid - IgG against hemidesmosomes, Eoisinophils w/in the blister
No oral involvement
Auspitz sign is
may also seen in this derm path unrelated to the skin?
pinpoint bleeding when the silver plaques are scraped off
pitting of the nails
puritic papules, vesicles and bullae in extensor surfaces after eating wheat product
Deposition of what?
dematitis herpatiformis in celiac
IgA in the tips of the dermal papillae
Erythema multiform is associated(4)
Can lead to ?
DRUGS
- sulfa,
- beta lactim, penicllin
- Anti-Seizure
- allopurinol
also
- infections (HSV, Mycoplasma pneumoniae)
- cancers
- Auto immune
Leads to Stevens Johnson Syndrome and TENS is >30% of the body
Presentation of Erythema multiforme
macules and papules forming a target lesion due to central necrosis
thought to be due to microvascular deposition of immune complexes
Stevens johnsons syndrome is different from Toxic epidermal necrolysis
Both have fever, bulla formation,necrosis, sloughing skin and high mortality
TENs has > 30% skin involvement
See a darkened skin in a flexor think of 2 things (neck or axilla)
diabetes mellatis
>40 gastrointestinal adenocarcinoma (Visceral malignancy)
Premalignant derm path treat w/ 5 florouricil
Found by?
actinic keratosis, treat due to squamous cell risk
found by feeling - sandpaper, rough
Painful lesion of the fat, commonly found on anterior tibia
Associated path (7)
erythema nodosum
sarcoidosis TB histioplasmosis coccidiomycosis Post strept leprosy Crohns
6 Ps of Lichen Planus
associated w/
purple Puriritic Papules polygonal shape Planar Plaque
Hepatitis C
-sawtooth infiltrate
see a herald patch on the neck and a couple days later see a christmas tree distribution of a rash called?
Rx
Pityriasis rosea
Self resolving in 6-8 wks
Necrotising fascititis caused by? (2)
suspect when? (2)
Clostridium perfenges and Strep pyrogenes
- When tenderness is beyond the erythematous border
- crepitis is felt
Honey rusted lesions of the superficial skin cause by (2)
Impetigo
Staph aureus
Strep pyrogenes
Erythematous painful lesion of the dermis and subcutaneous tissue caused by?(2)
Cellulitis
Staph aureus
Strep pyrogens
Exotoxin A and B from staph Aureus responsible for what derm path
pathophys?
Staph Scalded Skin Syndrome
keratinocyte attachment of stratum granulosum is destroyed -> sloughing skin
Pink pearly nodules caused by?
Keratin pearls caused by?
basal cell carcinoma
squamous cell carcinoma
most common malignant skin pathology
seen histology
basal cell carcinoma,
-Rarely METS locally destructive
palisading nuclei
Rolled birders, telanectasias and central necrosis
basal cell carcinoma
S 100 tumor marker important in
melanoma
Excisional biopsy key in prognosis of what disease
melanoma
- depth is key in risk of METs
Asymetrical
Borders (irregular)
Color (not uniform)
Diameter
Thickened scar around face or chest
keloid
Calcium deposition seen in a joint that is nonerrosive
acute pain and swelling?
Pseudogout
w. calcium pyrophosphate crystals seen on aspiration