Dermatopathology (Part 4 of 4) Flashcards

1
Q

What is mycosis fungoides?

A

a cutaneous T-cell lymphoma of CD4 T helper cells in the skin

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2
Q

how does mycosis fungoides present? (age, where, what do they look like)

A

usually >40 yo with truncal lesions; scaly red-brown patches; raised scaling plaques (can be confused with psoriasis); fungating nodules

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3
Q

eczema like lesions in patients with mycosis fungoides usually means what?

A

it is in the early stages

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4
Q

multiple tumor nodules in patients with mycosis fungoides usually means what?

A

systemic spread

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5
Q

what is sezary syndrome?

A

seen in some cases of mycosis fungoides; erythroderma, diffuse erythema and scaling of entire body surface

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6
Q

what is the morphology of sezary cells?

A

markedly folded nuclear membrane CEREBRIFORM contour

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7
Q

what is the histological hallmark of cutaneous t cell lymphoma?

A

the presence of atypical cells that characteristically form band-like aggregates within the superficial dermis and invade the epidermis as single cells and small clusters known as Pautrier microabscesses

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8
Q

what is urticaria pigmentosa?

A

a cutaneous form of mastocytosis that primarily affects children

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9
Q

how does urticaria pigmentosa present?

A

multiple, widely distributed lesions, round-oval, red-brown, nonscaling papule and small plaques; around 10% of cases have systemic disease with mast cells infiltrating organs (mostly seen in adults)

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10
Q

what is solitary mastocytoma?

A

usually seen in young kids; pink-tan-brown nodule with possible blister formation

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11
Q

many of the signs and symptoms of mastocytosis occurs when mast cells degranulate- what happens when mast cells degranulate?

A

histamine and heparin are released

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12
Q

what are two signs that can be seen with mastocytosis?

A

darier sign and dermatographism

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13
Q

what is darier sign?

A

localized area of dermal edema and erythema (wheal) when skin is rubbed

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14
Q

what is dermatographism?

A

area of dermal edema resembling a hive, result of local stroking skin with pointed instrument

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15
Q

what are the systemic symptoms associated with mastocytosis?

A

pruritus and flushing or bone pain

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16
Q

why is there bone pain seen in mastocytosis?

A

mast cell infiltration causes it

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17
Q

what could be a clue that a patient has mastocytosis?

A

if they are presenting with osteoporosis in premenopausal women or in men –> due to excessive histamine release in the bone marrow

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18
Q

what causes mastocytosis?

A

a point mutation in KIT receptor tyrosine kinase–> leads to mast cell growth and survival

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19
Q

what is the histological appearance of mastocytosis?

A

spindle shaped and stellate mast cells, fibrosis, edema, and few eosinophils

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20
Q

what stain can you use to diagnose mastocytosis?

A

giemsa (shows metachromatic granules)

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21
Q

what is ichthyosis?

A

impaired epidermal maturation, hyperkeratosis that results in a clinically fish-like scale appearance

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22
Q

what are three examples of acute inflammatory dermatoses?

A

urticaria, acute eczematous dermatitis, erythema multiforme

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23
Q

generally speaking, what are acute inflammatory dermatoses?

A

acute lesions that last from days to weeks that are characterized by inflammatory infiltrates (usually lymphocytes and macrophages) NOT NEUTROPHILS

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24
Q

generally speaking, what are chronic inflammatory dermatoses?

A

chronic lesions persist for months to years and are often associated with changes in epidermal growth or dermal fibrosis

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25
Q

what causes urticaria?

A

localized mast cell degranulation; it is an antigen induced release of vasoactive mediators from mast cells

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26
Q

what can urticaria cause?

A

wheals or angioedema

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27
Q

what is the common presentation of urticaria?

A

20-40 yo, less than 24 hours, sites exposed to pressure

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28
Q

how does urticaria look on a macro level?

A

small, pruritic papules to large edematous plaques; individual lesions may coalesce to form annular, linear, or arciform configurations

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29
Q

how does urticaria look on a micro level?

A

sparse superficial perivenular infiltrate of mononuclear cells (so around the veins)

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30
Q

which antibody is associated with urticaria?

A

IgE

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31
Q

what is one of the most common skin disorders?

A

acute eczematous dermatitis

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32
Q

what causes acute eczematous dermatitis?

A

external application of Ag or ingested food or drug

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33
Q

what are the 5 main causes of acute eczematous dermatitis?

A

allergic contact, atopic dermatitis, drug-related, photoeczematous dermatitis, primary irritant dermatitis

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34
Q

what is the official name of poison ivy/oak and what in it causes acute eczematous dermatitis?

A

Rhus toxicodendron- Urushiol

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35
Q

what is treatment of acute eczematous dermatitis?

A

it is palliative, no cure- just treat symptoms

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36
Q

what type of reaction is acute eczematous dermatitis?

A

T-cell mediated inflammatory reaction (type IV hypersensitivity)

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37
Q

how does acute eczematous dermatitis look on a macro level?

A

red, papulovesicular, oozing, and crusted (impetiginization) lesions

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38
Q

what do you get if acute eczematous dermatitis persists? (2 things leading to something)

A

you develop acanthosis and hyperkeratosis–> raised scaling plaques

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39
Q

what is spongiosis?

A

acute eczematous dermatitis, edema in the intracellular spaces, splaying them apart, particularly in the stratum spinosum

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40
Q

if eczematous dermatitis was set off by drugs, how might you tell?

A

there might be a lot more eosinophils present

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41
Q

what is erythema multiforme?

A

it is uncommon, self-limited hypersensitivity reaction to certain infections and drugs

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42
Q

How is erythema multiform characterized?

A

by keratinocyte injury mediated by CD8+ T lymphocytes

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43
Q

what is located in the center of the erythema multiform lesions? and the periphery?

A

CD8 cytotoxic T cells are in the center; CD4 T cells and Langerhans cells are in the periphery

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44
Q

what is the classic morphology of erythema multiforme?

A

targetoid lesions

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45
Q

what is interface dermatitis seen in erythema multiforme?

A

dermal edema, lymphocyte infiltration along the dermoepidermal junction, associated with dermal edema and degenerating keratinocytes

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46
Q

what are two important variants of erythema multiform to know about?

A

stevens-johnson and toxic epidermal necrolysis

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47
Q

what is stevens-johnson?

A

seen in children; extensive skin involvement plus oral mucosa, conjunctiva, urethra, genital, and perianal areas; secondary infections can lead to life-threatening sepsis

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48
Q

what is toxic epidermal necrolysis?

A

diffuse necrosis and sloughing of cutaneous and mucosal epithelia; resembles extensive burns- also life threatening

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49
Q

what are three examples of chronic inflammatory dermatoses?

A

psoriasis, seborrheic dermatitis, and lichen planus

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50
Q

what is psoriasis?

A

a chronic inflammatory dermatosis with an autoimmune basis; may also be asosciated with myopathy, and AIDS

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51
Q

what is the morphology of psoriasis?

A

pink to salmon colored plaque covered by loosely adherent silver scale

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52
Q

what is the Koebner phenomenon?

A

induce psoriatic lesions in susceptible patients by trauma, starts a self-perpetuating local inflammatory response

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53
Q

besides the skin what else can psoriasis affect and how?

A

the nails: pitting, dimpling, yellow-brown discoloration (oil slick); oncolysis (separation of the nail plate from the underlying bed)

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54
Q

what are the histological features of psoriasis?

A

marked acanthosis, regular downward elongation of rete ridges “test tubes in a rack”

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55
Q

what is munro microabscesses?

A

seen in psoriasis; when small PMN aggregates in parakeratotis stratum corneum

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56
Q

what is the Auspitz sign?

A

multiple, minute, bleeding points when the silver scale is lifted from the plaque

57
Q

there have been excellent clinical responses observed in patients with psoriasis when treated with what?

A

TNF and IL-17 inhibitors

58
Q

what is seborrheic dermatitis?

A

affects 5% of the population- more common than psoriasis; regions with increased sebaceous glans: scalp, forehead, external auditory canal; inflammation of epidermis; NOT A DISEASE OF SEBACEOUS GLAND

59
Q

what is seborrheic dermatitis associated with?

A

Parkinson’s disease; increased sebum production secondary to dopamine deficiency leads to increased seborrheic dermatitis; when you treat with levodopa–> there is a decrease in oil and improved seborrhea

60
Q

a severe form of seborrhic dermatitis that is difficult to treat was once common in what population of people?

A

HIV individuals with low CD4 counts; but incidence has fallen with the advent of effective antivrial therapy

61
Q

what is follicular lipping?

A

seen in cases of seborrheic dermatitis; mounds of parakeratosis containing neutrophils and serum are present at the ostia of hair follicles

62
Q

how can lichen planus be described?

A

pruritic, purple, polygonal, planar, papules, and plaques

63
Q

how do you treat lichen planus?

A

you don’t have to- it is self limited- resolves in 1-2 years; there is a post inflammatory hyperpigmentation left after resolution

64
Q

people with lichen planus are at increased risk of what?

A

squamous cell carcinoma

65
Q

koebner phenomenon is seen in what two disorders?

A

psoriasis and lichen planus

66
Q

what is wickham striae?

A

seen in lichen planus; papules highlighted with white dots, created by areas of hypergranulosis

67
Q

what is lichen planus/ how does it present? (not the 6 P’s, but what do the lesions look like and where?) 5 places

A

multiple symmetrical lesions especially on the extremities, wrists, elbows, and glans penis; 70% of cases have oral mucosal lesions

68
Q

what does lichen planus look like histologically?

A

dense cutaneous infiltrate of lymphs along the dermoepidermal junction (interface dermatitis); sawtoothing and CIVATTE bodies

69
Q

what are civatte bodies?

A

seen in lichen planus; they are anucleated necrotic basal cells incorporated into the inflamed papillary dermis

70
Q

what is the buzzword for lichen planus?

A

civatte bodies

71
Q

How can blistering (bullous) diseases be divided?

A

into inflammatory blistering disorders and noninflammatory blistering disorders

72
Q

what are the inflammatory blistering disorders?

A

pemphigus, bullous pemphigoid, and dermatitis herpetiformis

73
Q

what are the noninflammatory blistering disorders?

A

epidermolysis bullosa and prophyria

74
Q

bullous diseases produce dramatic lesions which can be fatal if untreated; what else is important to remember about diagnosing bullous diseases?

A

blisters occur at different levels of the skin; histologic assessment is essential for accurate diagnosis

75
Q

what is pemphigus?

A

when IgG autoantibodies disrupt intracellular lesions leading to blisters; there is a net-like pattern of intracellular IgG deposits

76
Q

what is the treatment for pemphigus?

A

immunosuppressives

77
Q

what is acantholysis?

A

seen in pemphigus; it is the dissolution of intercellular bridges connecting squamous epithelial cells

78
Q

what are the 5 different types of pemphigus?

A

vulgaris, vegetans, foliaceus, erythromatosus, and paraneoplastic pemphigus

79
Q

what is pemphigus vulgaris?

A

the most common form of pemphigus, it affects the mucosa and skin of the scalp, face, axilla, groin, trunk, and points of pressure ; the superficial vesicles rupture easily

80
Q

what is pemphigus vegetans? (where do you see it)

A

rare; large and wart like plaques studded with pustules; seen in the groin, axilla, and flexural surfaces

81
Q

what is pemphigus foliaceus?

A

benign, seen in people from brazil; seen on the scalp, face, chest, and back; erythema and crusting

82
Q

what is pemphigus erythromatosus?

A

localized less severe form of foliaceous; affects the malar area of the face (just like lupus)

83
Q

what is paraneoplastic pemphigus?

A

associated with malignancies such as NHL and lymphoid neoplasms

84
Q

what level does pemphigus foliaceus affect?

A

the stratum granulosum level- it is subcorneal- so it is right under the keratin at the top of your epidermis

85
Q

what level does pemphigus vulgaris affect?

A

above the basal layer- suprabasal- so it is above the basal layer- you’re going to retain those basal layer cells of the epidermis, and the rest of it is going to come off; if you have that much on top of it, it is going to be harder to rupture compared to the one that is subcorneal

86
Q

what layer does bullous pemphigoid affect?

A

subepidermal layer- the whole epidermis comes off

87
Q

what is the buzzword for the histology of pemphigus vulgaris?

A

tombstone appearance

88
Q

what is bullous pemphigoid?

A

seen in the elderly, typically on the inner aspects of thighs; flexor surfaces of forearms, axilla, groin, and lower abdomen; there are antibody deposits at the dermoepidermal junction

89
Q

what is dermatitis herpetiformis?

A

urticarial and grouped vesicles; bilateral and symmetrical grouped lesions on extensor surfaces of elbows, knees, upper back, and buttocks

90
Q

what is dermatitis herpetiformis associated with?

A

celiac disease (these people develop IgA antibodies against gluten) these antibodies cross react with reticulin to form a subepidermal blister

91
Q

how can you treat dermatitis herpetiformis?

A

remove gluten from the diet

92
Q

where is the blister for dermatitis herpetiformis?

A

subepidermal blister

93
Q

what antibody is associated with bullous pemphigoid?

A

IgG

94
Q

what antibody is associated with dermatitis herpetiformis?

A

IgA

95
Q

what is epidermolysis bullosa?

A

a noninflammatory blistering disorder; inherited defects in structural proteins; blisters at sites of pressure, rubbing, or trauma at or soon after birth

96
Q

what is porphyria?

A

a noninflammatory blistering disorder; a group of inborn or acquired disturbances in porphyrin metabolism

97
Q

what are porphyrins?

A

pigments normally present in Hgb, myoglobin, and cytochromes

98
Q

how does porphyria typically present?

A

urticaria and vesicles associated with scarring and exacerbated by sunlight

99
Q

where is the blister with epidermolysis bullosa?

A

subepidermal blister but NO INFLAMMATION

100
Q

what is rhinophyma and what is it associated with?

A

it is a permanent thickening of the nasal skin; associated with the 4th stage of rosacea

101
Q

what is rosacea associated with?

A

high cutaneous levels of antimicrobial peptide cathelicidin

102
Q

how do open comedones in acne vulgaris appear?

A

central black keratin plug

103
Q

how do closed comedones in acne vulgaris appear?

A

keratin plug trapped beneath the epidermal surface, potential for follicular rupture and inflammation

104
Q

what is the bacteria that causes acne?

A

propionibacterium acnes

105
Q

what is acne conglobate?

A

a severe variant of acne vulgaris, there is sinus tract formation and dermal scarring

106
Q

what is the treatment for P. acnes?

A

antibiotic for P. acnes; but also maybe isotretinoin (it has antisebaceous action)

107
Q

what is panniculitis?

A

inflammatory reaction in the subQ adipose tissue

108
Q

what are two forms of panniculitis?

A

erythema nodosum and erythema induratum

109
Q

what is erythema nodosum?

A

the most common form of panniculitis; it is poorly definied but exquisitely tender erythematous plaques and nodules

110
Q

what can be said about diagnosing erythema nodosum?

A

it can always be seen- you should palpate for it–> it feels ropy

111
Q

what type of reaction is erythema nodosum considered to be?

A

a delayed hypersensitivity reaction to microbial or drug related antigens

112
Q

what bacteria could cause erythema nodosum?

A

beta-hemolytic streptococcal infection

113
Q

what drugs could cause erythema nodosum

A

sulfonamides or oral contraceptives

114
Q

What is the uncommon type of panniculitis?

A

erythema induratum

115
Q

who does erythema induratum seem to affect primarily?

A

adolescents and menopausal females

116
Q

what exactly is erythema induratum?

A

primary vasculitis of deep vessels supplying fat lobules of the subQ–> leads to fat necrosis and inflammation

117
Q

how does erythema induratum present?

A

erythematous slightly tender nodule that usually ulcerates; early lesion: necrotizing vasculitis of small-medium arteries and veins in deep dermis and subQ

118
Q

how can erythema induratum be described? (what type of inflammation and what zones)

A

granulomatous inflammation and zones of caseous necrosis involving fat lobule

119
Q

What is verrucae?

A

warts; squamoproliferative disorders caused by HPV

120
Q

anogenital warts are caused by what strain(s) of HPV?

A

types 6 and 11

121
Q

what can HPV type 16 be associated with?

A

in situ SCC of the genitalia and with bowenoid papulosis

122
Q

what is bowenoid papulosis?

A

genital lesions of young adults with the histiologic appearance of carcinoma in situ, but which usually regress spontaneously

123
Q

what are HPV subtypes 5 and 8 associated with?

A

SCC, especially in individuals with epidermodysplasia verruciformis

124
Q

what is pathoneumonic for HPV? (on a micro level)

A

the Koilocytosis, which is just cytoplasmic vacuolization

125
Q

what can be seen insides warts?

A

keratohyaline granules

126
Q

what is molluscum contagiosum?

A

a common, self-limited poxvirus infection

127
Q

how does molluscum contagiosum look?

A

brick shaped, dumbbell shaped DNA core

128
Q

how does molluscum contagiosum present? (when, who, where, how do they feel, and how do they look)

A

usually after direct contact, children and young adults, trunk and anogenital regions; firm often pruritic, pink-skin colored umbilicated papules

129
Q

what is the buzz word for molluscum contagiosum?

A

the papules are UMBILICATED

130
Q

what does the histology of molluscum contagiosum show?

A

molluscum bodies: which is just the virus

131
Q

what is impetigo?

A

a common superficial bacterial infection of the skin; highly contagious; usually seen in otherwise healthy kids

132
Q

what causes impetigo?

A

staphylococcus aureus–> it has a toxin that causes epidermal injury (the toxin cleaves desmoglein 1)

133
Q

how does impetigo present?

A

as an erythematous macule initially, then multiple small pustules which break leaving shallow erosions; also a honey colored crust

134
Q

what happens if the honey colored crust is not fully removed from patients with impetigo?

A

new lesions form around the periphery and extensive epidermal damage may occur

135
Q

superficial fungal infections are confined to what skin layer?

A

stratum corneum

136
Q

tinea versicolor is a superficial fungal infection of what?

A

the upper trunk

137
Q

what causes tinea versicolor?

A

malassezia furfur- A YEAST

138
Q

how can you stain for tinea corpis and other superficial fungal infections?

A

using the PAS stain–> hyphae stains bright like magenta