5 dermatologic diseases Flashcards

1
Q

is desquamative gingivitis a diagnosis?

A

no, it is a clinical term to describe a manifestation of what is usually lichen planus, mucous membrane pemphigoid, or pemphigus vulgaris

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2
Q

clinical features of desquamative gingivitis

A
  • female predilection
  • diffuse, erythematous gingiva
  • involves attached tissues
  • sensitive to spicy food
  • may or may not be symptomatic
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3
Q

what is the most common lichenoid process condition we will see

A

idiopathic lichen planus

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4
Q

clinical features of lichen planus

A
  • purple, polygonal, pruiritic papules, very itchy

- flexor surfaces of extremities like wrists and shins

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5
Q

reticular form of lichen planus

A
  • more common than erosive or ulcerative
  • asymptomatic
  • posterior buccal mucosa
  • lacy white striations (wickham striae)
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6
Q

lacy white striations (wickham striae)

A

reticular form of lichen planus

-spiderweb like

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7
Q

lichen planus is usually bilateral or unilateral?*

A

bilateral

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8
Q

lichen planus most common form

A

reticular form

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9
Q

erthematous form of lichen planus

A
  • symptomatic
  • striae at periphery of erythema
  • may cause desquamative gingivitis (must be distinguished from MMP or pemphigus)
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10
Q

striae at periphery of erythema

A

erythematous form of lichen planus

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11
Q

least common form of lichen planus

A

ulcerative form

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12
Q

ulcerative form of lichen planus

A
  • symptomatic
  • white lesions, red lesions, + yellow lesions
  • ulcers with striae and erythema
  • may cause desquamative gingivitis (must be distinguished from MMP or pemphigus)
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13
Q

lichen planus is

A

idiopathic, don’t know why patients get them

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14
Q

lichen planus associated with what common medications

A
  • NSAIDS
  • anti-hypertensives
  • antihyperglycemics
  • cholesteral lowering agents
  • anti-hypothyroidism agents
  • antigout medications
  • amalgam restorations
  • cinnamon flavoring agents
  • herbal remedies
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15
Q

what is the exception to the rule that lichen planus is never unilateral

A

amalgam restorations or cinnamon flavoring if they only chew on one side of the mouth

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16
Q

Is lichen planus immune mediated?

A

yes, but not autoimmune bc it’s a T cell process

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17
Q

histopathologic features of lichen planus

A
  • orthokeratosis/parakeratosis that causes white wickam striae
  • saw tooth rete ridges
  • degeneration of basal cell layer
  • colloid bodies (Degenerative aptotic cells
  • band-like inflitrate of lymphocytes
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18
Q

easiest feature to recognize of lichen planus

A

squamitization of basal cell layer or bandlike infiltrate of lymphocytes

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19
Q

treatment of lichen planus

A
  • just observe if asymptomatic (don’t have to treat)

- topical corticosteroids

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20
Q

topical corticosteroids for treating lichen planus

A
  • fluocinonide or clobetasol gel (warn patient they should eat/drink for 30 min for longer contact time)
  • dexamethasone elixir
  • prophylactic antifungals in patients predisposed to candidiasis
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21
Q

malignant prognosis of lichen planus

A
  • needs life long follow up
  • 1-2 % increased risk of developing SCCA
  • reported cases associated with erosive form
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22
Q

lichen planus

A

wrist as purple polygonal papules, has fine white lines (Wickham’s striae)

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23
Q

erosive lichen planus

A

ulceratoin of buccal mucosa shows peripheral radiating keratotic striae

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24
Q

erosive planus often appears as

A

desquamative gingivitis, producing erythema + tenderness

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25
Q

what is almost indistinguishable from lupus?

A

lichen planus

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26
Q

3 subtypes of lupus erythematous

A

(autoimmune disease)
1 systemic lupus erythematous (SLE)
2 chronic cutaneous lupus eryhtematous (CCLE)
3 subacute cutaneous lupus eryhtematous

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27
Q

systemic lupus erythematous

A

-multisystem disease with skin + oral lesions

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28
Q

predilection os systemic lupus erythematous

A

8-10 : 1 females

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29
Q

clinical features of systemic lupus erythematous

A
  • fever, weight loss, arthritis, fatigue, malaise
  • 40-50% butterfly rash over malar area + nose
  • kidney involvement in 40-50%
  • cardiact involvement
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30
Q

clinical features in the mouth of clinical features of systemic

A
  • oral lesions in 5-40% of patients

- lichenoid lesions of palate, buccal mucosa, or gingiva (more likely than lichen planus to involve palate)

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31
Q

chronic cutaneous lupus erythematous oral clinical features

A
  • oral lesions identical to lichen planus
  • rarely without skin lesions
  • erythematous zone
  • surrounding fine white striae
  • possible central stippling white dots
  • may be sensitive to acidic or salty foods
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32
Q

subacute cutaneous lupus erythematous

A

features intermediate between SLE +CCLE

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33
Q

lupus

A

true autoimmune disease whereas lichen planus is not

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34
Q

ild SLE treatment

A

NSAIDs + antimalarials (antiinflammatory properties)

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35
Q

prognosis of SLE

A

95% 5 year survival
75% 15 year survival
renal failure= #1 cause of death

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36
Q

chronic cutaneous lupus eryhtematous skin lesions

A

scaling, atrophy, and pigmentary disturbances

-most evident on sun exposed skin

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37
Q

graft vs. host disease

A

-seen in allogeneic hematopoietic stem cell transplant recipients (8,000 annual transplants in USA)

38
Q

milder graft. vs. host disease seen in patients with

A
  • tend to do better
  • better histocompatibility match
  • younger age
  • cord blood received
  • female gender
39
Q

oral lesions clinical features of graft vs. host disease

A
  • sometimes, there’s only oral lesions
  • 33- 75% of acute patients, 80% of chronic patients
  • may be ONLY sign of disease
  • wickham striae
  • diffuse white papules on tongue, labial mucosa or buccal mucosa
  • burning mucosa (must rule out candidiasis)
40
Q

graft vs. host disease oral lesions

A
  • ulcers last >2 weeks
  • may relate to chemotherapy
  • r/o HHV or bacterial infection
  • xerostomia (tend to destry salivary glands)
  • palatal mucoceles
41
Q

people with graft vs. host disease have increased risk of

A

SCCA

42
Q

direct immunofluorescence

A

identifies autoantibodies bound to patient’s tissue

43
Q

indirect immunofluorescence

A

identifies autoantibodies circulating in patient’s blood

44
Q

when considering vesiculo

A
  • always include intact epithelium

- submit separate biopsies in 10% fomalin for H+ E or michel medium for DIF

45
Q

pemphigus related diseases

A
  • pemphigus vulgaris

- pemphigus vegetans

46
Q

pemphigus vulgaris

A

most common, if untreated, can be threatening

47
Q

pemphigus vegetans

A

rare, sometimes oral lesions

48
Q

pemphigus vulgaris- antibodies to

A

antibodies to desmosomes

49
Q

pemphigus vulgaris is a potentially life threatening

A

skin and mucous membrane disease

50
Q

clinical features of pemphigus vulgaris

A

-painful chronic oral sores, dysphagia, weight loss

51
Q

oral lesions of pemphigus vulgaris

A
  • 50% show oral lesions first, up to 1 yr before skin lesions
  • almost all show oral lesions eventually
  • erosions and ragged ulcerations of palate, gingiva, buccal, or labial mucosa
  • oral lesions often last to resolve
  • positive nikolsky sign
52
Q

histopathologic features of pemphigus vulgaris

A
  • perilesional tissue required for diagnosis
  • intraepithelial separation above basal layer (SUPRA basilar cleavage)
  • acantholysis (splitting apart) of spinous layer
  • generally positive with DIF IIF
53
Q

rounded tzanck cells

A

histologic features of pemphigus vulgaris

-keratinocytes lose some of their structure when they have acantholysis of spinous layer

54
Q

PV can be thought of like abrick wall

A

bricks precariously stay together, but with force they will fall apart

55
Q

tx of pemphigus vulgaris

A
  • corticosteroids
  • azathioprine
  • immunoglobulin therapy
  • untreated, mortality approaches 80%
56
Q

paraneoplastic autoimmune multiorgan syndrome (PAMS)

A
  • vesiculobullous lesions on skin + oral mucosa (patient can’t eat or swallow)
  • palmar + plantar bullae distinguish from pemphigus
  • crusted lips mimic erythema multiform
  • 70% have eye scarring
57
Q

crusted lips mimic erythema multiforme

A

paraneoplastic pemphigus autoimmune multiorgan syndrome

58
Q

paraneoplastic autoimmune multiorgan syndrome (PAMS) histopathologic features

A

sub-epithelial or intraepithelial clefting

59
Q

mucous membrane pemphigoid

A

autoimmune disorder with autoantibodies to hemidesmosomes, NOT DESMOSOMES***

60
Q

what is 2x as common as pemphigus vulgaris

A

mucous membrane pemphigoid

61
Q

average age of mucous membrane pemphigoid

A

50-60 years

-female predilection 2:1

62
Q

mucous membrane pemphigoid

A
  • only ocular involvement
  • only oral cavity involvement
  • mucosal + cutaneous lesions
  • multiple mucosal surfaces, no significant skin disease
63
Q

mucous membrane pemphigoid

A

postiive nikolsky sign **

64
Q

clinical features of mucous membrane pemphigoid

A

oral lesions seen in most, but conjunctiva, nose, esophagus, larynx, and vagina may be invovled

65
Q

25% with oral lesions in mucous membrane pemphigoid may develop

A

eye lesions

66
Q

mucous membrane pemphigoid histopathologic features

A

sub-epithelial separation below basal layer (SUB basilar cleavage)

67
Q

mucous membrane pemphigoid treatment

A
  • topical steroids for oral lesions

- refer to opthalmologist

68
Q

trichiasis

A

eyelashes rubbing aginst the eye itself

-upper eyelid turns inward

69
Q

bullous pemphigoid

A

similar to MMP (mucous membrane pemphigoid) but with very rare oral lesions

70
Q

epidermolysis bullosa acquisita

A

50% of patients have oral lesions (most with cutaneous lesions)

71
Q

linear IgA disease

A

50% have mucosal involvement

72
Q

erythema multiforme is triggered by

A

cross reactivity to herpes simplex virus

  • rapid onset
  • immune mediated condition
73
Q

erythema multiforme most cases are secondary to

A

infection; typically HSV or mycoplamsa pneumoniae

74
Q

predilection for erythema multiforme

A

male predilection; mostly ages 20-40

75
Q

erythema multiforme is one of few conditions that can present with

A

rash on palms of hand (papilloma fabe syndrome does as well)

76
Q

clinical features of erythema multiforme

A
  • erythematous mucosal patches
  • shallow ulcers (very painful)
  • hemorrhagic crusts of lips**
  • entire perimeter of tongue may be affected
  • targeteoid cutaneous lesoins
77
Q

targeteoid cutaneous lesoins

A

looks like a bulleye

78
Q

**common clinical features of erythema multiforme

A

hemorrhagic crusts of lips + targetoid cutaneous lesions

79
Q

erythema multiforme treatment

A

systemic and or topical steroids

80
Q

stevens-johnson syndrome

A
  • milder form of toxic epidermal necrosis
  • usually result of medication
  • mucocutaneous disease often confused with erythema multiforme
  • stevens-johnson syndrome invovles head and trunk and is triggered by a drug reaction rather than an infection
81
Q

stevens-johnson syndrome affects

A

ocular or genital mucosa involved in conjunction with oral and skin lesions

82
Q

toxic epidermal necrolysis aka

A

lyell disease

83
Q

toxic epidermal necrolysis

A

severe form of stevens-johnson syndrome which involves more than 30% of total body surface area

84
Q

age of most patients with toxic epidermal necrolysis

A

> 60 years

85
Q

toxic epidermal necrolysis diffuse sloughing of

A

a significant portion of skin and mucosal surfaces, predisposing to dehydration and infection

86
Q

toxic epidermal necrolysis management

A

as for burn patients

87
Q

toxic epidermal necrolysis mortality rate

A

25-30%

88
Q

oral manifestations of dermatologic diseases lichenoid diseases

A
  • idiopathic lichen planus
  • lichenoid drug eruptions
  • lupus erythematosus
  • graft vs. host disease
89
Q

oral manifestations of dermatologic diseases- autoimmune diseases

A
  • pemphigus vulgaris
  • paraneoplastic autoimmune multiorgan syndrome
  • mucous membrane pemphigoid
90
Q

oral manifestations of dermatologic diseases-immune mediated diseases

A
  • erythema multiforme

- stevens-johnson syndrome/toxic epidermal necrolysis