Path I: Skin Lecture 4

Front 1

what is langerhans cell histiocytosis?

Back 1

histiocytosis ~ general for proliferative disorder of dendritic cells/ mphage

Front 2

what is present in the cytoplasm of Langerhans on EM?

Back 2

Birbeck granules (tennis racket)

Front 3

how do langerhans appear under LM?

Back 3

convoluted, vesicular nuclear mbr with linear folds

Front 4

immunohistochem methods to ID langherhans cells?

Back 4

CD1a and S-100

Front 5

clinical presentation: langerhans histiocytosis

Back 5

papules, nodules, or erthymatous scaling plaques

Front 6

unifocal and multifocal unisystem langerhans cell histiocytosis

Back 6

eosinophilic granuloma
bone lesion or pulmonary lesion
nonlethal

Front 7

what is a cutaneous T cell lymphoma arising in the dermis?

Back 7

mycosis fungoides

Front 8

what is the prognosis for mycosis fungoides?

Back 8

malignant, fatal

Front 9

2 types of mycosis fungoides

Back 9

mycosis fungoides: chronic, proliferative over yrs
mycosis fungoides d'emblee: nodular, eruptive; progresses rapidly

Front 10

what are Sezary cells? what do they cause?

Back 10

malignant T cells that enter leukemic phase in mycosis fungoides; cause sezary syndrome: diffuse erythema and scaling

Front 11

who gets mycosis fungoides? and where?

Back 11

>40 yo; peaks >60
men>women
blacks>whites?asians
trunk and extremities

Front 12

prognosis of mycosis fungoides

Back 12

slow progression
fatal over 10-20 yrs
improved with tx

Front 13

3 phases of mycosis fungoides

Back 13

chronic dermatitis
patch stage
plaque stage

Front 14

chronic dermatitis phase of mycosis fungoides

Back 14

flat discoloration
(early patch)
starts growing in dermis so initially flat

Front 15

patch stage of mycosis fungoides

Back 15

raised, indurated, irregular erythematous patch/plaque
redder

Front 16

plaque stage of mycosis fungoides

Back 16

ulceration or dark nodules suggest systemic spreading (hemtogenous)
scaling

Front 17

histo mycosis fungoides

Back 17

epidermaltrophism:
atypical lymphocytes slowly accumulate in superficial dermis
eventually infiltrate epidermis as single cells,clusters ->swell to form microabscesses

Front 18

mastocytosis: defn

Back 18

rare clonal neoplasm of mast cells in skin

Front 19

who is affected in mastocytosis and how does it usually present?

Back 19

children- local cutaneous form
Urticaria Pigmentosa-
mult, oval, red-brown scaling? papules
infants-solitary mastocytoma

Front 20

what causes the s/s in mastocytosis?

Back 20

effects of histamine:
flushing rxn in resp to foods etc
pruritis-itching
nosebleeds (heparin)

Front 21

histo: mastocytosis

Back 21

ovoid cells
uniform, central nucleus
dermis
Giemsa stain for metachromatic granules

Front 22

ichthyosis: defn

Back 22

genetic disorders
fish-like scales
hyperkeratosis

Front 23

3 types of ichthyosis and mode of inheritance

Back 23

ichthyosis vulgaris- AD; acquired due to prolonged rubbing
lamellar ichthyosis- AR
X-linked ichthyosis

Front 24

histo: ichthyosis

Back 24

compact stratum corneum-hyperkeratosis

Front 25

urticaria: defn and charac (who,where, tx)

Back 25

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

Front 26

presentation: urticaria

Back 26

erythematous, edematous plaques = wheals
blanch with direct pressure

Front 27

histo: urticaria

Back 27

normal epidermis
edematous papillary dermis
dilated vasculature

Front 28

acute eczematous dermatitis: defn and presentation

Back 28

general: acute onset
red, papulovesicular lesions
"boiling over"
may ooze, crust; pruritic
evolves into raised, scaly plaques

Front 29

eczema: who and tx

Back 29

kids
tx: topical corticosteroids

Front 30

stages of eczema (4)

Back 30

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

Front 31

classifications of eczematous dermatitis (6)

Back 31

contact dermatitis-topical
atopic dermatitis-unknown
drug-rel eczematous dermatitis-systemic
eczematous insect bite
photoeczematous rxn-UV
primary irritant dermatitis-repeat trauma, chemical irritant

Front 32

histo: eczematous dermatitis

Back 32

intercellular edema widens intercellular spaces in epidermis-> fluid-filled intraepidermal vesicles
prominent intercellular bridges

Front 33

mxn allergic contact dermatitis

Back 33

langerhans cells process/present Ag
sensitization and re-exposure

Front 34

erythema multiforme: defn

Back 34

HSR to drugs, infection, malignancy, collagen vascular disease

Front 35

erythema multiforme: presentation

Back 35

array (multiform) of lesions: macule, papule, vesicle, bullae

TARGET LESION- red macule, papule with pale or vesicular center of epidermal necrosis

Front 36

what is steven-johnson syndrome?

Back 36

severe systemic febrile form of erythema multiforme
can be lethal
assoc with antibiotics

Front 37

histo: erythema multiforme

Back 37

lymphocytes collect at dermoepidermal jxn (~T cell lymphoma)
edematous papillary dermis
necrotic keratinocytes

Front 38

psoriasis: defn and charac (where, how many)

Back 38

chronic inflamm dermatosis
1-2% pop
elbow,knees scalp, lumbosacral region, intergluteal cleft, glans penis

Front 39

lesion in psoriasis

Back 39

well demarcated, pink plaque covered in lose silver scales

Front 40

clinical assoc with psoriasis

Back 40

arthritis (mild to severe); resembles rheumatoid arthritis

Front 41

progession of psoriasis

Back 41

early: eruptive lesions; inflamm and erythema
chronic: erythema covered by thick silver scale

Front 42

histo: psoriasis

Back 42

incr keratinocyte turnover
acanthosis (thick epidermis)
deep rete pegs
thin stratum granulosum
thick parakeratotic scale
dilated vessels
PMNs throughout

Front 43

lichen planus: defn

Back 43

lichen(thickened) planus(flat)
self-limiting
chronic inflamm of skin and mucous mbr (oral)
resolves spontaneously 1-2 yrs

Front 44

lesions: lichen planus

Back 44

pruritic, purple, polygonal, papules
flat topped papules->plaques
white dots (wickham striae)
wrist, elbows, glans penis

Front 45

lichen planus: histo

Back 45

saw-toothed dermoepidermal border
dense lymphocyte infiltration at border makes it vague
thickened granular layer and kyperkeratosis

Front 46

discoid lupus erthematosus: defn

Back 46

SLE with only skin symptoms: malar erythema across bridge of nose
discoid plaques

Front 47

chronic plaques in DLE

Back 47

thinned, atrophic epidermis
central hypopigmentation
peripheral hyperpigmentation
dilated, tortuous vessels

Front 48

histo:DLE

Back 48

lymphocytes infiltrate in papillary and reticular dermis
thinning of epidermis
loss of rete pegs
hyperkeratosis

Front 49

direct immunoflourescence in DLE

Back 49

IgG and C' positive Band test at dermoepidermal jxn