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49 Cards in this Set

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what is langerhans cell histiocytosis?
histiocytosis ~ general for proliferative disorder of dendritic cells/ mphage
what is present in the cytoplasm of Langerhans on EM?
Birbeck granules (tennis racket)
how do langerhans appear under LM?
convoluted, vesicular nuclear mbr with linear folds
immunohistochem methods to ID langherhans cells?
CD1a and S-100
clinical presentation: langerhans histiocytosis
papules, nodules, or erthymatous scaling plaques
unifocal and multifocal unisystem langerhans cell histiocytosis
eosinophilic granuloma
bone lesion or pulmonary lesion
nonlethal
what is a cutaneous T cell lymphoma arising in the dermis?
mycosis fungoides
what is the prognosis for mycosis fungoides?
malignant, fatal
2 types of mycosis fungoides
mycosis fungoides: chronic, proliferative over yrs
mycosis fungoides d'emblee: nodular, eruptive; progresses rapidly
what are Sezary cells? what do they cause?
malignant T cells that enter leukemic phase in mycosis fungoides; cause sezary syndrome: diffuse erythema and scaling
who gets mycosis fungoides? and where?
>40 yo; peaks >60
men>women
blacks>whites?asians
trunk and extremities
prognosis of mycosis fungoides
slow progression
fatal over 10-20 yrs
improved with tx
3 phases of mycosis fungoides
chronic dermatitis
patch stage
plaque stage
chronic dermatitis phase of mycosis fungoides
flat discoloration
(early patch)
starts growing in dermis so initially flat
patch stage of mycosis fungoides
raised, indurated, irregular erythematous patch/plaque
redder
plaque stage of mycosis fungoides
ulceration or dark nodules suggest systemic spreading (hemtogenous)
scaling
histo mycosis fungoides
epidermaltrophism:
atypical lymphocytes slowly accumulate in superficial dermis
eventually infiltrate epidermis as single cells,clusters ->swell to form microabscesses
mastocytosis: defn
rare clonal neoplasm of mast cells in skin
who is affected in mastocytosis and how does it usually present?
children- local cutaneous form
Urticaria Pigmentosa-
mult, oval, red-brown scaling? papules
infants-solitary mastocytoma
what causes the s/s in mastocytosis?
effects of histamine:
flushing rxn in resp to foods etc
pruritis-itching
nosebleeds (heparin)
histo: mastocytosis
ovoid cells
uniform, central nucleus
dermis
Giemsa stain for metachromatic granules
ichthyosis: defn
genetic disorders
fish-like scales
hyperkeratosis
3 types of ichthyosis and mode of inheritance
ichthyosis vulgaris- AD; acquired due to prolonged rubbing
lamellar ichthyosis- AR
X-linked ichthyosis
histo: ichthyosis
compact stratum corneum-hyperkeratosis
urticaria: defn and charac (who,where, tx)
acute allergic rxn-emergency (may be fatal)
severe discomfort, pruritic
age: 20-40
trunk, extremities
usually resolves 24 hrs; may last days, months
tx: subQ epinephrine and corticosteroids
presentation: urticaria
erythematous, edematous plaques = wheals
blanch with direct pressure
histo: urticaria
normal epidermis
edematous papillary dermis
dilated vasculature
acute eczematous dermatitis: defn and presentation
general: acute onset
red, papulovesicular lesions
"boiling over"
may ooze, crust; pruritic
evolves into raised, scaly plaques
eczema: who and tx
kids
tx: topical corticosteroids
stages of eczema (4)
1. dermal edema, inflamm cells
2. epidermal spongiosis (accumulation of fluid in intercellular spaces of epidermis) and microvesicle formation
3. scales and epidermal hyperplasia, parakeratosis
4. hyperkeratosis and fibrosis
classifications of eczematous dermatitis (6)
contact dermatitis-topical
atopic dermatitis-unknown
drug-rel eczematous dermatitis-systemic
eczematous insect bite
photoeczematous rxn-UV
primary irritant dermatitis-repeat trauma, chemical irritant
histo: eczematous dermatitis
intercellular edema widens intercellular spaces in epidermis-> fluid-filled intraepidermal vesicles
prominent intercellular bridges
mxn allergic contact dermatitis
langerhans cells process/present Ag
sensitization and re-exposure
erythema multiforme: defn
HSR to drugs, infection, malignancy, collagen vascular disease
erythema multiforme: presentation
array (multiform) of lesions: macule, papule, vesicle, bullae

TARGET LESION- red macule, papule with pale or vesicular center of epidermal necrosis
what is steven-johnson syndrome?
severe systemic febrile form of erythema multiforme
can be lethal
assoc with antibiotics
histo: erythema multiforme
lymphocytes collect at dermoepidermal jxn (~T cell lymphoma)
edematous papillary dermis
necrotic keratinocytes
psoriasis: defn and charac (where, how many)
chronic inflamm dermatosis
1-2% pop
elbow,knees scalp, lumbosacral region, intergluteal cleft, glans penis
lesion in psoriasis
well demarcated, pink plaque covered in lose silver scales
clinical assoc with psoriasis
arthritis (mild to severe); resembles rheumatoid arthritis
progession of psoriasis
early: eruptive lesions; inflamm and erythema
chronic: erythema covered by thick silver scale
histo: psoriasis
incr keratinocyte turnover
acanthosis (thick epidermis)
deep rete pegs
thin stratum granulosum
thick parakeratotic scale
dilated vessels
PMNs throughout
lichen planus: defn
lichen(thickened) planus(flat)
self-limiting
chronic inflamm of skin and mucous mbr (oral)
resolves spontaneously 1-2 yrs
lesions: lichen planus
pruritic, purple, polygonal, papules
flat topped papules->plaques
white dots (wickham striae)
wrist, elbows, glans penis
lichen planus: histo
saw-toothed dermoepidermal border
dense lymphocyte infiltration at border makes it vague
thickened granular layer and kyperkeratosis
discoid lupus erthematosus: defn
SLE with only skin symptoms: malar erythema across bridge of nose
discoid plaques
chronic plaques in DLE
thinned, atrophic epidermis
central hypopigmentation
peripheral hyperpigmentation
dilated, tortuous vessels
histo:DLE
lymphocytes infiltrate in papillary and reticular dermis
thinning of epidermis
loss of rete pegs
hyperkeratosis
direct immunoflourescence in DLE
IgG and C' positive Band test at dermoepidermal jxn