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125 Cards in this Set

  • Front
  • Back
A soccer player who was kicked in the leg suffered a damaged medial meniscus. What else has been damaged?
ACL- unhappy triad
A gymnast who dislocates her shoulder anteriorly. What nerve has been damaged
Axillary- C5,C6
An X-ray shows bilateral hilar lymphadenopathy
What is the diagnosis?
Sarcoid
Child has weakness and enlarged calves. What is the disease and how is it inherited?
Duchenne's
X-linked
25 y/o woman w/ low-grade fever, rash across her nose; wide spread edema. What disease are you concerned about?
SLE
85 y/o man w/ acute knee pain and swelling.
X-ray shows joint space w/o erosion. What is the diagnosis? What is seen on aspiration?
pseudogout
rhomboid calcium pyrophosphate crystals
What are the layers of the epidermis from surface to base?
Californians Like Girls in String Bikinis
Corneum
Lucidum
Granulosa
Spinosa
Basalis
What are the components of the epithelial cell junction?
Zona occludens- tight junction
Zona adherens- intermediate junction- has actin filaments and E-cadherin
Macula adherins (Desmosome)- small discrete sites of attachment- Desmoplakin and keratin
Gap Junction- adjacent cells to communicate for electric and metabolic functions
Hemidesmosome- integrin
How is bone formed?
intramembranous- spontaneous bone formation w/o preexisting cartilage
Endochondral- Ossification of cartilagenous molds. Long bones form by this type of ossification at 1ry and 2ry centers.
What is in the Unhappy triad?
ACL- lateral to medial; attaches anteriorly to tibia
PCL- medial to lateral; attaches posteriorly to tibia
Medial collateral, and lateral collateral on the sides

clipping on the lateral side gives you damage to the MCL, ACL and medial meniscus

+ drawer sign- ACL
Abnl passive abduction- MCL
What are the muscles of the rotator cuff?
SItS
Supraspinatus- helps deltoid abduct the arm
Infraspinatus- laterally rotates the arm
teres Minor- adducts adn laterally rotates the arm
Subscapularis- medially rotates and adducts the arm.
What is the cycle of musculoskeletal contraction?
conformation of the myosin head is altered w/ loss of P. Bond between actin and myosin is increased.

release of ADP causes a shift in the architecutre-> thin filament is pulled toward the sarcomere

Bond between myosin and actin is broken w/ ATP

Myosin head is attached
ATP is hydrolysed, weak bond between actin and myosin forms
What is the role of Ca in muscle?
binds to troponin C-> conformational change.
causes tropomyosin to move out of the way to allow actin/myosin cycling
How does teh action potential function in skeletal muscle?
ap reactions axon terminal-> depolarizes and opens voltage gated Ca channels and causes neurotransmitter vesicle fusion and exocytosis. Postsynaptic ligand binding leads to depolarization of the postsynaptic muscle cell.

Depol travels down T tubule. Dihydropyridine receptor, voltage-sensing Ca channel protein. lies in the T-tubule membrane next to a ryanodine receptor, which lies in the sarcoplasmic reticulum

Released Ca binds to troponin C, whcihc moves tropomyosin out of the way, Myosin hydrolyzes ATP and is displaced-> power stroke

Contraction -> HIZ shrinkage
A band is constant
How does smooth muscle contract?
ap-> smooth muscle membrane depolarization-> voltage-gated Ca channels open-> increased Ca in cytoplasm-> ca binds to calmodulin-> activates myosin light-chain kinase; triggers phosphorylatio of myosin
Mosin light-chain phosphatase removes P.
What is the difference between disuse atrophy and denervation atrophy?
denervation- involves both type I and type II fibers; small and angular
target fibers
Reinnervation- fiber-type grouping; cluster of type I fibers adjacent to a cluster of type II fibers, instead of a mixture

Disuse- angular atrophy, type II fibers
Duchenne muscular dystrophy
mos commona nd most severe of the muscular dystrophies
- male
- weakness in proximal muscles of the extremities at 1y/o; immobalization and waisting, death in tens
- histo: random variation in muscle fiber size, necrosis of individual muscle fibers, and replacement of necrotic fibers by fibrofatty tissue
- increased serum CK
presents in proximal muscles of the extremities
Compensatory hypertrophy of distal sites, calf muscles, followed by pseudohypertrophy. Increased fibrous tissue and adipose tissue.
deficiency of dystrophin
X-linked inheritance w/ many de novo mutations. Various deletions or point mutations occur-> DNA frameshift, stop codons and failure of synthesis.
Becker muscular dystrophy
Becker is Better
clinically similar to Duchenne
abnl, truncated dystrophin, segmental deletions, no frameshifts. less functonal
Faciocapulohumeral muscular dystrophy
AD inheritance
associated w/ slowly progressive, nondisabling course and an almost normal life expectancy
Sequentially involves the muscles of the face, scapular area, and humerus
Limb-girdle dystrophy
AR inheritance
proximal muscles of the shoulder, pelvic girdle, or both
Myotonic dystrophy
AD
increasing CTG repeats
weakness w/ myotonia (can't relax once contracted)
cataracts and testicular atrophy and baldness
central core disease
flobby baby
normal CK
- loss of mitochondria and other organelles in type I muscle fibers
- muscle weakness an hypotonia, but infants can become ambulatory
nemaline myopahty
tangles of small rod-shaped granules predominantly in Type I
varies clinically from a mild nonprogressive disease to severe wekaness ending in death from resp failure
Mitochondrial myopathies
non-mendelian
ragged red appearance of muscle fibers.
Kearns-Sayre syndrome- ophthalmoplegia, pigmentary retinopathy, heart block, cerebellar ataxia.
Myesthania Gravis
auto immune against ACh receptors
muscle weakness that increases w/ use
ptosis or diplopia, difficulty chewing, speaking, or swallowing
complicated by resp failure

Thymic tumors, hyperplasia, 3x more in women
Lambert-Eaton
paraneoplastic disorder
defect in release of ACh
Osteoporosis
decrease in bone mass
- impared synth or increased reorp of bone matrix
- bone structures inadequate for weight bearing.-> kyphosis and short

- post menopausal- egen deficienc
- physical inactivigty
- hypercorticism
- hyper thyroid
- calcium deficiency

type 1- post menopausal
type 2- senile > 70
W>black>asian
crushc fractures-> acute back pain, loss of height, kyphosis
distal radius (Colles') fractures
vertebral wedge fractures

Bisphos or pulsatile PTH
Osteitis fibrosa cystica
von Recklinhausen disease
hyperPTH
wisdespread lesions
Brown tumor- cystic spaces lined by multinucleated osteoclasts and filled w/ vasc fibrous stroma often w/ brown discoloration from hemorrhage
diffuse radiolucency of bone mimicking osteoporosis
lab manifestations of hyperPTH
high Ca, low P, and high alk phos
Osteomalacia
Vit D defiicency
defective calcification of osteoid matrix
increased PTH, increased serum phosphate; reversible w/ vit D
2ry to renal disease-> renal osteodystrophy
Rickets
Vit D deficiency in kids
decreased Ca and excess accumulation of osteoid, leading to increased thickness of the epiphyseal growth plates and other skeletal deformities

Craniotabes- thinning and softening of occipital and parietal bones
Late closing of fontanelles
Rachitic rosary- thickening of the costochondral junctions that results in a string-of-beads
Harrison groove- depression along the line of insertion of the diaphragm inot the rib cage
Pigeon breast- protrusion of the sternum
Decreased height- spinal deformity
Paget disease
osteitis deformans
elderly
unknown etiology; viral etiology is suggested by ultrastructural, intranuclear inclusions in osteoclasts
Abnl bone architecture casued by increases in bone architecture. increased osteoblastic and osteoclastic activity
spine, pelvis, calvarium, femur, tibia
Abnl bone architecture caused by increase in both osteoblastic and osteoclastic activity.
Serum Ca, phosphorus, PTH levels are normal. Serum alk phos is elevated
What are the morphologic phases of paget's?
osteolytic- osteoclastic resorption predominates
Mixed osteoblastic and osteolytic phase- new bone formation- mosaic
late phase- bone density is increased, thick trabeculae; mosaic pattern is prominent
What are the complicatiosn of pagets
Bone pain from fractures- bone is thick but lacks strength
High-output cardiac failure- can result from multiple functional arteriovenous sunts w/in highly vascular early lesions
Hearing loss- narrowoing of the auditory foramen or direct involvement of the bones of the middle ear.
Osteosarcoma- 1% of cases; otherwise seen in young people.
Scurvy
Vit C deficiency
bone lesions from impared osteoid matrix formation
failure of proline and lysine hydroxylation needed for collagen synth
- Subperiosteal hemorrhage (painful)
- Osteoporosis (metaphyseal ends of bone)
- Epiphyseal cartilage not replaced by osteoid
Achondroplasia
one of the most common causes of dwarfism
AD disorder caused by a mutation in the fibroblast growth factor receptor 3, located at 4p16
short limbs w/ normal head and trunk
chaaracterized by narrow epiphyseal plates and bony sealing off of the area between the epiphyseal plate and the metaphysis-> short thick bones.
Monostotic fibrous dysplasia
solitary lesions that are often asymptomatic, spontaneous fractures w/ pain, severe deformity
Polyostotic fibrous dysplasia
Multiple sites are involved, it can be associated w/ severe deformity
Fibrous bone disease
McCune-Albright
Polyostotic fibrous dysplasia, precocious puberty, cafe-au-lait spots on skin and short stature, very young girls
Aseptic necrosis
unknown etiology
infarction from interuption of arterial blood supply
2ry to trauma, embolism, decompression syndrome "the bends" and sickle cell.

in growing children
- femur (legg-Calve-Prthes disease)
- Tibial tubercle (Osgood-schlatter disease)
- navicular bone (Kohler bone disaese)
Osteogenesis imperfecta
brittle bone disease
caused by a group of specific gene mutations, all result in defective collagen synthesis, results in generalized CT abnlties, affect teeth, skin, eyes, and bones.
blue sclerae from translucency of thin CT overlying the choroid
- occurs in a variety of clinical types varying greatly in severity; AD dominant
multiple childhood fractures are prominent clinical findings.
Osteopetrosis
marble bone disease, Albers-Schonberg
- increased density of the skeleton
osteoclastic activity
- mult fractures in spte of increased bone density
anemia as a result of decreased marrow space
blindness, deafness, cranial nerve involvement because of narrowing and impingement of neural foramina
- 2 clinical forms: AR - fatal, less severe AD

normal Ca, P, and alk phos
Pyogenic osteomyelitis
Kids- blood-boarn Staph, mos common
newborns- Group B Strep or E. coli
Salmonella- sickle cell
Adults- compound fracture or durgery
IV drugs- pseudomonas

acute pyogenic- meaphysis; distal endo fthe femur; proximal end of the tibia; proximal end of the humorus

may resolve
may compress vasculature w/ pyogenic exudate, resulting in ischemic necrosis of the bone and marrow. Nectoric bone is a foreign body, locus for persistent infection
Subperiosteal dissection by pyogenic exudate can impare blood supply; pus can rupture and form sinuses draingn through the skin.
a sleeve of new bone formation may surround the infected necrotic area
localized by a surrounding wall of granulation tissue (Brodie abscess)
2ry reactive systemic amyloidosis
TB osteomyelitis
Vertebrae- vertebral collapse- spinal deformity
Hip
Long bones, femur and tibia
Bones of the hands and feet
Histiocytosis X
various sites
prolif of histiocytic cells; birbeck granules, tennsi racket cytoplasmic structures
Letterer-Siwe disease
acute disseminated langerhans cell histiocytosis
agressive, fatal disorder of infants and small kids
hepatosplenomegaly, lymphadenopathy, pancytopenia, pulm involvement, recurrent infections as a result of widespread histiocytic prolif
Hand-Schuller-christian disease
better prognosis
before 5 y/o
histiocytic proliferation mixed w/ inflammatory cells in bone, espicially in the skull, liver, spleen, and other tissues
- skull lesions, Diabetes Insipitus and exophthalmos w/ orbit invovlement
Eosinophilic granuloma
best prognosis of the histiocytosis
- solitary bone lesion; extraskeletal invovlement is limited to lung
histiocytic prolif mixed w/ inflamm cells, macros, lymphos, eos
Osteochondroma
bone growth covered by a acap of cartilage projecting form the surface of a bone
most common benign tumor of bone
occurs most frequently in men younger than 25 years of age
hamartoma rather than a true neoplasm
originates from metaphysis of long bones w/ lower end of the femur or on the upper end of the tibia being favored locations
- rarely undergoes transition to chondrosarcoma; malignant transformation is more frequent in multiple familial osteochondromatosis, rare hereditary variant characterized by mult lesiosn
Giant cell tumor
oval or spindle-shaped cells intermingled with numerous multinuclear giant cells
- epiphyseal end of long bones- 50% occur about the knee
- soap bubble on radiograph
- peak incidence in 20-40
M>W
locally aggressive and recurs
Osteosarcoma
-most common 1ry malignant tumor of bone
- peak in men 10-20
metaphysis of long bones; proxima lportion of the tibia and most distal portion of th efemur are preferred sites

- Pain and swelling and ocasionally path fracture
- increased alk phos
- lifting of the periosteum by the expanding tumor, Codman triangle; sunburst pattern of growth.
- early spread to lungs, liver, and brain
What are the predisposing factors to Osteosarcoma
Pagets, fibrous dysplasia, chondroma, osteochondroma
Ionizing radiation
Bone infarcts
Familial retinoblastoma; Rb on 13
Chondrosarcoma
malignant cartilaginous tumor
peak in men 30-60
may arise as 1ry tumor or from transformation of pre-existing cartilaginous tumors, especially multiple familial osteochondromatosis or multiple enchondromatosis
- has a characteristic sites of origin incluiding the pelvis, spine, or scapula; proximal humerus or proximal femur; and femur or tibia near the knee.
Ewing Sarcoma
anaplastic small blue cell malignant tumor
looks like malignant lymphoma
occurs in long bones, ribs, pelvis, and scapula
Peak incidence in boys younger than 15 yrs of age
- follows an extremely malignant course w/ early mets
responds to chemo
early stages-mimics acute osteomyelitis
- 11; 22 translocation
- onion-skin appearance
Rheumatoid arthritis
chronic inflamm disorder of synovial joints
women aged 20-50.
autoimmune, presence of RF, IgM w/ anti IgG Fc specificity
HLADR4
What is the morphology of rheumatoid?
synovitis
- acute inflammatory reaction w/ edema and inflamm infiltrate beginning w/ neutrophils and followed by lymphocytes and plasma cells

-Hyperplasia and hypertrophy of the synovial lining-> finger like villi

-Granulation tissue- pannus; extends over articular cartilage; extension of pannus to subchondral bone results in erosion and cyst formation, deformities of obth cartilage and bone

-Scarring contracture and deformity from destructive inflam of ligaments, tendons, and bursae

- subcutaneous rheumatoid nodules
what is the clinical course of rheumatoid?
Episodic change
- fatigue, malase, anorexia, weighth loss, fever, myalgia
- swelling of the joints, stiffness, in the morning or after inactivity
- poly articular and symmetric joint involvement

Chronic joint changes
- PIP and MP joints are sites
- ulnar deviation
- minimal radial deviation of the wrist

Extra-articular- Pleural and pericardial effusions
anemia of chronic disease
vasculitis
lymphadenopathy
pulm involvement
neurologic abnlties
2ry reactive amyloidosis
What are the variants of rheumatoid?
Sjogren syndrome- w/ rheumatoid
Felty syndrome: splenomegaly, neutropenia and RA
Still disease (juvenile) preceded or accompanied by generalized lymphadenopathy and hepatosplenomegaly, marked fevere
What are the spondyloarthropathies
No rheumatoid
HLA B27
Peripheral arthritis
Sacroiliitis
- Ankylosing spodylitis, Reiter, Psoriatic, IBD
Ankylosing spondylitis
HLA-B27
Spine and sacroiliac joints-> rigidity and fixation of the spine as a result of bone fusion
Reiter syndrome
Reiter syndrome- urethritis, conjunctivitis and arthritis- venereal or intestinal infection
Psoriatic arthritis
10% of pts w/ psoriasis
IBD arthritis
peripheral arthritis or ankylosing spondylitis w/ UC or Crohns
Osteoarthritis
chronic inflamm joint disease characterized by degeneration of articular cartilage w/ new bone formation subchondrally and at the margins of the affected joint
most common form of arthritis
high inscidence in women
mechanical trauma to the affected joints- "wear-and-tear arthritis"
What are the characteristic morphologic change of Osteoarthritis?
loss of elasticity, pitting and fraying of cartilage; fragments may spearate and float into synovial fluid

Eburnation: polished, ivory-like appearance of bone, from erosion of overlying cartilage
Cystic changes in subchondral bone
New bone formation
- increased density of subchondral bone
- osteophyte- formation a the perimeter of the articular surface and at points of ligamental attachement to bone

Osteophyte fracturing adn floating into synovial fluid- joint mice;
Heberden nodes: osteophytes at the DIP joints
Bouchar proximal interphalanges
What is the difference between 1ry and 2ry osteoarthritis?
1ry- w/o known cause; genetics, inflamm
2ry- mechanical, metabolic factors cause it. (ochronosis)
Gout
deposition of urate crystals in several tissues especially the joints as a result of hyperuricemia
- intense inflamm reaction beginning w/ opsonizaitno of crytstals by IgG, followed by pahgocytosis by neutrophils and eventuating in the release of proteolytic enzymes and inflamm mediators from phagocytic cells
- manifest by an inflamm response that leads to extremely painful acute arthritis and bursitis
- meatarsophalangeal joint of the great toe Podagra
Large meal or alcohol-> hyper uricemia
nodular tophi
helix and the anti heliz of the ear, Achilles and other sites.

1ry- hyperuricemia w/o cause.
middle-aged men
familial

2ry
Leukemia, MM, and Myeloproliferative syndromes w/ increased cell turnover
Decreased urate excretion because of chronic renal failure
Lesch-Nyhan syndrome- hyperuricemia w/ severe neuro manifestations- self-mutilation (x-linked)
thiaziedes

(-)birefringement
Chondrocalcinosis (pseudogout)
calcium pyrophosphate dihydrate crystal deposition
Inflamm reaction in cartilage
resembles gout
basophilic rhomboid crystals
+ weak birefringement
How do you treat Gout?
allopurinol
probenecid, colchicine and NSAIDS
Hypertrophic osteoarthropathy
systemic disorders- chronic lung disease, congennital cyanotic heart disease, cirrhosis of the liver, IBD
- clubbing of the fingers, periostitis
Lupus
women (80%); child-bearing age
ANA, immune complex inflamm lesions

wireloop lesiosn in kidney

Fever, malaise, lymphadenopathy and weight loss
Joints- arthralgia and arthritis
Skin rashes - butterfly
Raynaud- vasospasm of small vessels; fingers
Serosal inflammation - pericarditis and pleuritis
Diffuse interstitial pulm fibrosis- pneumonitis or fibrosing alveolits
Endocarditis- libman-Sacks; mitral valve
Immune complex vasculitis- vessels of any organ. Splenic arterioles from onion -skin appearance
Glomerular chagne- subendo immune complex
Neuro and psych manifestations
Eye changes, yellowish, cotton wool-like fundal lesions
Lupus
I'M DAMN SHARP
Immunoglobulin
Malar Rash
Discoid rash
Antinuclear Ab
Mucositis
Neurologic
Serositis- pleuritis, pericarditis
Hematologic
Arthritis
Renal
Photosensitivity
What labs are seen in SLE?
LE test- mechanically damaged neutrophils; autoantibody serum
+ in 70%
+ANA
decreased compliment
dermal-epidermal immune complexes
False + syphilis test
Scleroderma
Widespread fibrosis and degenerative changes that affect skin, GI, heart, muscle, and lung, kidney
young women
anti-Scl70

presents w/ skin changes, polyarthralgias, esophageal symps

Hypertrophy of collagen
Sclerodactyly
Raynaud
VViceral organ- esophagus, interstitial pulm fibrosis
HTN
CREST
Calcinosis
raynaud
Esophageal dysfunction
Sclerodactyly
Telangiectasia
Sjogren syndrome
women in late middle age
triad- xerostomia, keratoconjunctivitis sicca, and oneof several CT or other autoimmune diseases
- SLE, PSS, Polymyositis, Hoshimoto
- Sicca syndrome- only drymouth and conjunctivitis; nasal vaginal dryness, bronchitis, reflux esophagitis

Salivary glands- bilateral enlarged parotids diffulely infiltrated by lymphocytes and plasma cells. This cell infiltration can partly or compltely obscure the parenchyma
can invole the lacrymal glands

SS-A (ro), SS-B (la), hypergammaglobulinemia
Polymyositis
Chronic inflammatory process especially involving the proximal muscles of the extremities
CD8 T-cell induced injury

dermatomyositis- purple rash on the face and neck
Increased CK, aldolase, postiver ANA anit-Jo-1
Mixed CT disease
mainly women- peak incidence at 35-40
Shares clinical features w/ other connective tissue disorders, but in contrast renal involvement is rare.
arthralgias, Raynauds, esophageal hypomotility and myositis, myalgias, fatigue
specific ANAs
anti-nRNP/ U1RNP
Polymyalgia rheumatica
Pain and stiffness in shoulders and hips, often w/ fever, malase and weight loss
no muscular wekness
increased ESR
occurs in pts > 50. Temporal arteritis
prednisone
Celiac sprue
autoimmune-mediated intolerance of gliadin leading to steatorrhea
northwern european.
blunding of villi
lymphocytes in the lamina propria, abnl D-xylose test.
Jejunum.
Dermatitis herpetiformis
10-15%-> malignancy (T-cell lymphoma)
Goodpastures
pulm hemorrhages-> hemptysis
renal lesions-> hematuria, anemia, crescentic glomerulonephritis
anti- BM disease

theare are 2 Good Pastures for this disease: Glomerulus and Pulmonary
type II hypersensitivity
Sarcoidosis
Immune-mediated-widespread
noncaseating granulomas and elevated serum ACE levels. COmmon in black females.

Restrictive lung disease, bilateral hilar lymphadenopathy, erythema nodosum
Bells palsy, epithelial granulomas containing microscopic Schaumann and asteroid bodies, uveoparotitis, and hypercalcemia (elevated converstion of vit. D to its active form in epithelioid macrophages

GRIAN
Gammaglobulinemia
Rheumatoid arthritis
ACE increase
Interstitial fibrosis
Noncaseating granulomas
Ankylosing spondylitis
chronic inflamm disease of spine and sacroiliac joints-> ankylosis, uveitis, and aortic regurge
Reiter's
Triad
Urethritis
Conjunctivitis and anterior uveitis
arthritis

Can't see, can't pee, can't climb a tree
post-Gi or chlamydia infections
Eczematous dermatitis
heterogeneous group of pruritic inflam disorders
Infection
Chemicals- contac dermatitis- Ags in type IV cell-mediated hypersensitivity reactions resulting from cooperation of skin of macrophages

Atopy- allergy; type I anaphylactic-type; hay fever or bronchial; skin is thype IV not type I

Acute- spongiosis w/ vesicle formation
Chronic- acanthosis, hyperkeratosis, lichenification; focal lymphocytic dermal infiltrates
Subacute- intermediate changes between acute and chronic;
less spongiosis and vesiculation than in acute; less acanthosis and hyperkeratosis than in chronic eczematous dermatitis
Neurodermatitis
Lichen simplex chronicus
clinically indistinguishable from chronic eczematous dermatitis
- anatomic changes from scratching
Psoriasis
- chronic inflamm from erythematous papules and plaques w/ characteristic silvery scaling; lesions are sharply demarcated
- most often involves the extensor surfaces of the elbows and knees as well as the scalp and sacral area
- non pruritic
histo: epidermal prolliferation w/ acanthosis and highly characteristic parakeratosis; minute neutrophilic abscesses (Munro abscesses) may be found w/in the parakeratotic stratum corneum
may be of autoimmune etiology
- can be associated w/ severe destructive rheumatoid arthritis-like lesions (psoriatic arthritis) that most commonly affect teh fingers
Knees and elbows
increased spinosum
decreased graulosum
Auspitz sign
Varicella
viral infection- fever and vesicular eruption
immune may have herpes zoster
may remain latent for years in dorsal root ganglia
painful skin eruption of herpes zoster has a characteristic distribution along the dermatomes corresponding to the affected dorsal root ganglia
Pemphigus vulgaris
people aged 30-60
acantholytic disorder characterized by formation of severe intraepidermal bullae. First lesions often occur in oral mucosa and extensive skin involvement follows. Lesions often rupture, leaving large denuded surfaces to secondary infection
- prominent intraepidermal acantholysis and sparing of the basal layer.
- autoimmune disorder characterized by IgG autoAbs directed against the epidermal intercellular cement; can be fatal
Immunofluorescense
Bullous Pemphigoid
less severe than pemphigus
subepidermal bullae, characteristic inflamm infiltrate of eosinophils in the surrounding dermis

autoimmune disorder characterized by IgG autoAbs directed against epidermal Basment Membraine. Linear band of immuno fluorescense

Spares oral mucosa
Dermatitis herpetiformis
20-40,
recurrent pruritic blistering disorder, usually of the extensor surfaces of the knees, elbows, scalp, upper back, and sacral area. Blisters occur in groups

- dermal microabscesses w/ neutrophils and eosinophils at the tips of dermal papillae which become subepidermal blistsesr
- demonstrates deposits of IgA at the tips of dermal papillae
Commonly associated w/ gluten sensitive enteropathy
both skin lesiosn and enteropathy improve when patients are on gluten free diets.
Erythema multiforme
multiple types of lesions, macules, papules, and vesicles
- target lesion
hypersensitivity- malig, autoimmune, drug
Stevens-Johnson syndrome is the major form of erythema multiforme. High fever, bulla formation and necrosis, ulceration of the skin, high mortality rate
Lichen planus
pruritic, purple, polygonal papules; infiltrate of lymphocytes at dermoepidermal junction
Albinism
failure of pigment production
Ocular- melanin dysfunction of the eye- X-linked

Oculocutaneous- eyes, skin hair; predisposes to actinic keratosis, basal and squamous carcinoma, and melanoma
- tyrosinase-negative- failure of tyrosine conversion
- tyrosine positive- unknown mechanism
Vitiligo
Aquired loss of melanocytes; autoimmune
associated w/ graves, addison
Nevocellualr and blue nevi
Nevocellular- benign tumor or hamartoma; melanocyes
Junctional- nevus cells confided to the epidermal-dermal junction
compoud- junction and in the nevus
intradermal- confined to clusters w/in the dermis

Blue nevus- at birth; nodular foci of dendritic highly pigmented melanocytes in the dermis; dermal=blue
spitz nevus
juvenile
benign
spindle shaped cells
Dysplastic nevus
atypical, irregularly pigmented lesion w/ disorderly proliferation of melanocytes, dermal fibrosis and often subjacent dermal lymphocytic infiltration
- may transform into malignant melanoma
- familial in some cases; AD inheritance and a marked tnedency toward conversion to malignant melanoma
Lentigo maligna
precursor to melanoma
irregular macular pigmented lesion on sun-exposed skin
Molluscum contagiosum
contagious viral disorder in children and adolescents. direct contact
DNA pox virus
umbilicated, dome-shaped papules
Verruca vulgaris
benign papilloma
HPV, not the same as gyne neoplasms
vacuolated cells (koilocytes)
Actinic keratosis
premalignatn from sunlight
Acanthosis nigricans
marker of visceral malig
acanthosis and hyperpigmentation
Seborrheic keratosis
common bengn neoplasm of older persons
raised papules w/ pasted on appearance; head, trunk and extremities
Keloid
abnl prolif of CT; deranged arrangement of collagen fibers, large raised tumor-like
Trauma; ear piercing
recur after resection
genetics
african
Squamous cell carcinoma
locally invasive, rare mets
sunlight, face, back of the hands. from pre-existing actinic keratosis
chemical carcinogens, arsenic, rads or x-ray
invasion of dermis by sheets and islands of neoplastic epidermal cells w/ keratin pearls
Basal cell carcinoma
most common of all skin tumors
sun-exposed areas. head and neck
upper part of the face
grossly presents as a pearly papule.
telangiectatic vessels
clusters of darkly staining basaloid cells w/ typical palisade arrangement of the nuclei
locally aggressive, ulcerate, bleed, never mets
surgical resection
Malignant melanoma
Growth phases
Radial
- growth in all directions, but predominantly lateral w/in the epidermis and papillary zone of the dermis
- lymphocytic response is prominnet
- melanomas in the radial growth phase do not mets;, cure is frequent

Vertical
- growht extends into reticular dermis or beyond.
prognosis varies w/ depth
lymphatics or hematogenous mets may occur
Clincial variants of melanoma
Lentigo malgina melanoma- sun-exposed skin. Radial growth phase predominates initially; most often develops from preexisting lentigo maligna

Superficial spreading melanoma- most common variat. lesion is irregularly bordered w/ variegated pigmentation; most frequent on the trunk and extremities. Radial growth phase predominates

Nodular melanoma- vertical growth phase, poor prognosis

Acral-lentiginous- hands and feet of dark-skinned persons
Polyarteritis nodosa
necrotizing immune complex of inflam
small and medium sized arteries
- destruction of arterial media and internal elastic lamina aneurysmal nodules
- HBV
- fever, weight loss, malaise, ab pain, headache, myalgia, HTN
- serum Abs to neutrophilic myeloperoxidase
P-ANCA
Kidneys- vasculitis in the arterioles and glomeruli; renal lesions and HTN cause most deaths from polyarteritis nodosa
Coronary arteries from ischemic heart disease
Musculoskeletal system-> myalgia, arthralgia, arthritis
GI tract-> nausea, vomiting or abdominal pain
CNS, ey or skin

Rx- coritcosteroids, cyclophosphamide
Churg-Strauss
necrotizing vasculitis; variant of poly-artertitis nodosa
prominent involvement of the pulm vasculature; marked peripheral eosinophilia
asthma
Henoch-Schonlein purpura
young kids
poststrep
hemorrhagic urticaria
extensor surfaces of arms, legs, and buttocks; fever arthralgias and GI and reanl involvement
URIS
Serum Sickness
generalized deposition of th AG-Ab complexes
Wegener granulomatosis
necrotizing granulomatous vasculitis; small to medium sized vessels of respiratory and kidney
Paranasal and lungs, necrotizing glomerulonephritis
fibrinoid necrosis of small arteries and veins, early infiltration by neutrophils
Granuloma formation w/ giant cells is prominent
circulating anti-neutrophil cytoplasmic antibodies w/ a cytoplasmic staining pattern
Takayasu's arteritis
pulseless diease
granulomatous thickening of aortic arch
proximal great vessels
Fever
Arthritis
Night sweats
MYalgia
SKIN nodules
Ocular distrubances
Weak pulses in upper extremities
medium and large arteries
Buerger's disease
thromboangiitis obliterans
idiopathic, segmental thrombosing vasculitis of intermediate and small peripheral arteries and veins
heavy smokers
Intermittent claudication, superficial nodular phlebitis, cold sensitiviety, severe pain in affected part
may cause gangrene

quit smoking
Temporal arteritis
most common vasculitits of medium and small arteries
branches of carotis
focal granulomatous
unilateral headache, jaw claduication, impaired vision

1/2 have systemic polymyalgia rhematica
increased ESR
- steroids
Opiod toxicity
addiction, resp depression, constipation, miosis, additive CNS depression w/ other drugs
No tolerance to mosis and constipation
Toxicity treated w/ naloxone
Colchicine
acute gout
depolymerizes microtubules impairing leukocyte chemotaxis and degranulation.
GI SEs if given orally
Probenecid
chronic gaout, inhibs reabsorp of uric acid
Allopurinol
chronic gout
inhibs xanthine oxidase,
less conversion to uric acid
used in lymphoma and leukemia to prevent tumor lysis
etanercept
recombinant TNF
infliximab
TNF-Ab
Cyclosporine
binds to cyclophililns. COmplex blocks the diff and activation of T-cells by inhibiting calcineurin. Prevents the production of IL-2 and its receptor
Tacrolimus
binds to FK-binding protein inhibiting secretion of IL-2 and other cytokines
Potent immunosuppresive used in organ transplant recipients

nephrotox, peripheral neuropathy, HTN, pleural effusion
hyperglycemia
Azathioprine
Antimetabolite derivative of 6-mercaptopurine that interferes with the metabolism and synth of nucleic acids. Toxic to proliferating lymphocytes

Kidney transplant

SE bone marrow suppresssion. Active metabolite is metabbed by xanthine oxidase. allopurinol should not be give with this