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92 Cards in this Set

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subepi bullae with eosinophil inflamm infiltrate in surrounding dermis =
bullous pemphigoid; IgG autoAb against epi BM--get characteristic IF band along BM
dermal microabscesses with PMNs, eos @ tips of dermal papillae and IgA dep=
dermatitis herpetiformis
ocular albinism
X linked; melanin dysfunction limited to eyes
types of oculocutaneous albinism
tyrosinase(-)-failure of conversion of tyrosin to DOPA (intermed. in melanin synthesis); tyrosinase(+)--mech unknown
aquired LOSS of melanocytes in discrete areas; autoimmune?
blue nevus
present at birth; nodular foci of dendritic highly pigmented melanocytes in DERMIS
Spitz nevus
benign! but often confused with malignant melanoma; in kids
skin tag
benign neoplasm that presents as firm nodule, characterized by intertwining bundles of collagen and fibroblasts
dermatofibrosarcoma protuberans
slow growing, well-diff'd malignant neoplasm that histologically resembles dermatofibroma (intertwining bundles of collagen and fibroblasts)
acanthosis nigrans sometimes marker of?
visceral malignancy
variants of capillary hemangioma
port wine stain; strawberry hemangioma--bright red raised; cherry hemangioma--small dome shaped papule
Sturge-Weber syndrome
port wine stain on face + ipsi glaucoma + vascular lesion of ocular choriodal tissue + extensive hemangiomatous involvement of meninges
granuloma pyogenicum
vascular pedunculated lesion characterized by numerous capillaries and edematous stroma; often develops following trauma
histo characteristics of BCC
darkly staining basaloid cells with typical palisade arrangement of nuclei of cells at periphery of tumor cell cluster
nodular melanoma
begins in vertical growth phase!! very poor prognosis
disuse atrophy assoc'd with
angular atrophy, mostly type II fibers
death in Duchenne's muscular dystrophy most commonly due to?
pneumonia caused by weakness of respiratory muscles
facioscapulohumeral muscular dystrophy
AD, slowly progressive but nondisabling with almost normal lifespan; mm of face then scapular then humerus
myotonic dystrophy
AD, CTG repeat in myotinin protein kinase gene (nl<30 repeat); myotonia, cataracts, testicular atrophy, male pattern baldness
central core dz
congenital myopathy (nl sr CK levels); loss of mitochondria et al in central portion of type I fibers--> weakness and hypotonia but babies eventually ambulatory
nemaline myopathy
congenital myopathy (nl sr CK levels); tangles of small rod-shaped granules mostly in type I fibers; varies clinically
Kearns-Sayre syndrome
a mitochondrial myopathy (ragged red fibers); ophthalmoplegia, pigmentary retinopathy, heart block, cerebellar ataxia; (only from mama!)
Lambert-Eaton syndrome
paraneoplastic (SCLC!); acquired Ab that reacts with presynaptic V-gated calcium channels
von Recklinghausen dz of bone
osteitis fibrosa cystica ("brown tumor"); due to primary or secondary hyperparathyroidism-->widespread osteoclastic lesions
clinical manifestations of Rickets
craniotabes (thin/soft parietal/occipital bones); late closing of fontanelles, rachitic rosary, Harrisons groove (depression along line of instertion of diaphragm into ribcage); pigeon chest
complications of Paget dz (increased activity of o'blasts and o'clasts)
bone pain (fx); high output cardiac failure; hearing loss; osteosarcoma
3 types of fibrous dysplasia
monostotic--usually asympt but can have spontaneous fx; polyostotic; McCuneAlbright-polyostotic with precocious puberty, cafe au lait spots, short stature (young girls)
Osgood-Schlatter dz
avascular necrosis of tibial tubercle
Legg-Calve-Perthes dz
avascular necrosis of femoral head
Kohler dz
avascular necrosis of navicular bone
3 eponynomous avascular necroses in children
Legg-Calve-Perthes--head of femur; Korhler--navicular; Osgood-Schlatter--tibial tubercle
osteopetrosis--due to? assoc'd with?
(marble bone dz); due to failure of o'clastic activity; assoc'd with anemia (dec'd BM); blindness deafness CN involvement (narrowing and impingment of neural foramina)
bugs causing pyogenic osteomyelitis in a)kids b)newborns c)sickle cell d)IV drug users
a)staph aureus; b)gp B beta strep, e.coli; c) salmonella; d) pseudomonas
Brodie abscess
wall of granulation tissue seen in pyogenic osteomyelitis
Letterer-Siwe dz
acute disseminated Langerhans cell histiocytosis; aggressive, usually fatal of babies; hepatosplenomegaly, lymphadenopathy, pancytopenia, pulm involvement , recurrent infections
Hand-Schuller-Christian dz
chronic progressive histiocytosis; triad: skull lesions, DI, exophthalmos (orbit involvement)
variants of histiocytosis X
Letterer-Siwe dz (v bad); Hand-SChuller-Christian dz (not as bad); eosinophilic granuloma (best prognosis)
most common benign bone tumors
osteochondroma-hamartoma, Ring of Ranvier defect; giant cell-"soap bubble" on XR, locally aggressive
2-3xAP increase + Codman triangle + sunburst pattern =
osteosarcoma predisposing factors
Paget dz, fibrous dysplasia, chondroma, osteochondroma; ionizing radiation; bone infarcts
"small blue cell" tumor + t(11;22) =
Ewing sarcoma (in early stages may mimic acute osteomyelitis)
HLA type assoc'd with RA?
Felty syndrome
RA, splenomegaly, neutropenia
Still dz
juvenile RA
triad of Reiter syndrome
urethritis, conjunctivitis, arthritis
Heberden nodes vs Bouchard nodes
(both osteophytes in OA); Heberden--DIP of fingers; Bouchard--PIP of fingers
most common soft tissue sarcomas of late middle-old age?
malignant fibrous histiocytoma
malignant tumor of fibroblasts; spindle shaped cells in herringbone pattern
spina bifida
failure of posterior verterbral arches to close
TORCH complex
Toxoplasmosis, Other, Rubella, CMV, HSV; --> microcephaly, focal cerebral calcification
hydrocephalus ex vacuo
hyydrocephalus without obstruction or increase in CSF production; seen in d/o with dec'd cerebral mass (e.g. Alzheimers)
external hydrocephalus
increased CSF confined to subarachnoid space
Arnold-Chiari malformation
downward displacement of cerebellar tonsils and medullar thru foramen magnum; --> hydrocephalus; also usually thoracolumbar meningomyelocele
tuberous sclerosis syndrome
AD, nodular proliferation of mulitnucleated atypical astrocytes; adenoma sebaceum of skin; "ash leafe spots"; angiomyolipoma of kidney; seizures and mental retardation beginning in infancy
most frequent site of CVA--thrombotic vs embolic?
thrombotic occlusion--carotid bifurcation, MCA; embolic--MCA
lacunar strokes clinical manifestations
purely sensory(thalamus) or motor(internal capsule)
Charcot Bouchard aneurysms
minute dilations at small artery bifurcations in HTN; may rupture and cause intracranial hemorrhage
intracranial hemorrhage most often where?
basal ganglia/thalamus (most often due to HTN)
lymphatics in CNS
no lymphatics enter CNS
hydrocephalus and pyogenic meningitis?
meningitis can cause reactive fibroblastic arachnoiditis wiht scarring and obliteration of subarachnoid space--> hydrocephalus
CSF findings in bacterial meningitis
increased PMNs, increased proteins, decreased glucose
most frequent cause of bacterial meningitis in a)infants/newbies b)children/young adults c) older adults
a)listeria, gp B beta strep, e.coli; b) strep pneumo, n.meningitides; c)strep pneumo, GNR
most common source of cerebral abscess
middle ear
periventricular calcification in newborn, think?
toxoplasmosis (or CMV)
how is CSF in viral meningitis different from bacterial meningitis?
viral--inc'd lymphocytes, relatively increased protein, NORMAL GLUCOSE; bacterial--inc'd PMNs, inc'd protein, DECREASED glucose
degeneration and necrosis of anterior horn cells of spinal cord
small cysts in CNS gray matter with striking absence of inflammatory response=
prion dz
lack of M compoment of measles virus--
subacute sclerosing panencephalitis
Charcot triad
nystagmus, intention tremor, scanning speech; often a clinical manifestation of MS
acute disseminated encephalomyelitis
post infectious myelitis (delayed hypersensitivity?); widespread demyelination
albumino-cytologic dissociation of CSF--
very increased [protein], modest increase in cell count; seen in Guillain-Barre syndrome
morphologic abnormalitis in Alzheimers
neurofibrillary tangles; senile plaque; granulovaculor deg'n (w/i pyramidal cells of hippo); amyloid deposition; Hirano bodies (actin inclusions); generalized cerebral atrophy
E4 allele of apoprotein E
(csome 19) occurs with greater frequency in Alzheimers pts
choline acetyltransferase deficiency
seen in Alzheimers
significance of nucleus basalis of Meynert?
decreased neurons wthin this nucleus assoc'd with Alzheimers
Binswanger dz
assoc'd with HTN; multiple lacunar infarcts, progressive demyelination limited to subcortical area with characteristic sparing of cortex; subcortical leukoencephalopathy
atrophy of striatum (caudate nucleus and putamen) seen in?
HD (CAG repeat)
genetics of HD
AD, CAG repeat with huntingtin gene on csome 4
Shy-Drager syndrome
parkinsonism with autonomic dysfxn and orthostatic hypotension
progressive bulbar palsy
brainstem and cranial nerve involvement leads to difficulty swallowing, speaking; ends in respiratory failiure
most frequent cause of conjunctivitis?
adenovirus infection
retrolental fibroplasia
retinopathy of prematurity; O2 toxicitiy--> blindness
retinitis pigmentosa
hereditary night blindness with progressive loss of central vision due to loss of rods, then cones; retinal pigmentation seen on ophthalmoscopy
most common form of glaucoma?
open angle (gradullay increasing IOP)
increase in IOP on dilation of pupils=?
closed angle glaucoma
most common primary intracranial tumors in a) adults b) kinds
a) glioblastoma multiforme, meningioma, acoustic neuroma; b) cerebellar astrocytoma; medullloblastoma
"pseudopalisade" arrangment of tumor cells seen in?
glioblastoma multiforme
Name the tumor: slow growing, closely packed "fried egg" cells, foci of calcification
name the tumor: rosettes, blepharoplasts (rod shaped structures near nuclear representing basal bodies of cilia), may cause hydrocephalus
ependymoma (usually in 4th ventricle)
histology of meningioma
whorled pattern of concentrically arranged spindle cells and laminated calcified psammoma bodies
2 patterns of schwannoma
Antoni A--interlacing bundles of elongated cells with palisading nuclei; Antoni B--looser, less cellular