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PATH II > skin and mucosa dx 2 > Flashcards

skin and mucosa dx 2 Flashcards

1
Q

Lichen planus
 Commonality? affects what tissues?
 dx type?
 Lichenoid mucositis?

A

 Common, chronic disease that affects skin and oral mucosa
 Immune mediated mucocutaneous disorder
 Medications may cause similar appearance: lichenoid mucositis

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2
Q

Lichen planus
 Clinical features:
 MC in?
 Skin lesions:
 Often affect?

A

 MC in middle-aged female adults
 Skin lesions: purple, pruritic, polygonal, papules (4-P’s)
 Often affect flexor surfaces of extremities

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3
Q

Lichen planus
 Clinical features of Reticular type:
common?
 what oral sites?
 app?
 Post-inflammatory?
 symptoms?

A

most common type
 Involves buccal mucosa bilaterally
 Interlacing white lines – Wickham striae
 Wax and wane
 Post-inflammatory melanosis
 Usually asymptomatic

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4
Q

Lichen planus
 Clinical features of Erosive type:

A

 Atrophic, erythematous areas with central ulceration
 Patients often symptomatic
 Periphery bordered by fine, white radiating striae
 Atrophy and ulceration confined to gingiva: desquamative gingivitis

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5
Q

Lichen planus
 Diagnosis:

A

 Clinical, histopathology, direct immunofluorescence
 10% Formalin for 95% biopsies vs Michels solution for DIF samples

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6
Q

Lichen planus tx (reticular vs erosive)

A

 Reticular: usually asymptomatic, no tx needed
 Erosive: topical corticosteroids

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7
Q
A

reticular LP

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8
Q
A

reticular LP

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9
Q
A

post inflam melanosis
LP

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10
Q
A

erosive LP

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11
Q
A

erosive LP

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12
Q
A

erosive LP

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13
Q

where else could LP lesions be?

A

gingiva and tongue

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14
Q

how does LP present on the tongue?

A

non-specific white plaques

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15
Q
A

LP

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16
Q

lichen planus histo

A

Lymphocytic infiltrate at rete pegs with linear fibrinogen in DIF

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17
Q

pt also presents with keratitotic webbing pattern in oral mucosa

A

Lichen planus

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18
Q

DIF + for fibrinogen

A

lichen planus

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19
Q

Erythema multiforme
 def? etiology?
 Likely an?
 50% of cases have?

A

 Ulcerative mucocutaneous condition of uncertain etiology
 Likely an immune mediated process
 50% of cases: precipitating cause – infections (ie: herpes simplex), medications (infrequently)

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20
Q

Erythema multiforme
 Clinical features:
 Often observed in what age group?
 Prodromal symptoms?
 lesion app?

A

 Often observed in young adults (20s and 30s)
 Prodromal symptoms: Fever, malaise, headache, cough
 Slightly elevated, round, dusky-red patches on skin
 May appear as concentric circular erythematous rings – target lesion

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21
Q

Erythema multiforme
 Clinical features:
most frequently involved mucosal site?
 other mucosal sites?
 Oral lesions? where?
 lips?

A

 Oral cavity: most frequently involved mucosal site
 Ocular, genitourinary, respiratory mucosa may be affected
 Oral lesions: shallow erosions or ulcerations with irregular borders (Lips, labial mucosa, buccal mucosa, tongue, FOM, soft palate)
 Hemorrhagic crusting of the vermilion zone of lips

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22
Q

Erythema multiforme minor/major differences?
severe ocular involvement?

A

 Erythema multiforme minor: milder cases
 Skin lesions and 1 mucosal site (usually oral)

 Erythema multiforme major: more severe
 Widespread skin lesions and 2 or more mucosal sites

 Severe ocular involvement: scarring (symblepharon formation)

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23
Q

Erythema multiforme tx

A

 Usually self-limiting (2-6 weeks)
 Systemic or topical corticosteroids

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24
Q
A

erythema multiforme

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25
Q
A

erythema multiforme

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26
Q
A

erythema multiforme

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27
Q

skin lesions present as well with concentric rings

A

erythema multiforme

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28
Q

skin lesions with ringed app present as well

A

erythema multiforme

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29
Q

Stevens – Johnson syndrome and Toxic epidermal necrolysis

A

 Severe blistering diseases triggered by drug exposure
 SJS: <10% skin and mucosal involvement
 TEN: >30% skin and mucosal involvement

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30
Q

SJS/TEN clinical features
 SJS: usually seen in?
 TEN: usually seen in?
 Initially present with?
 After a few days what appears? app?
 May have what changes as well?

A

 SJS: usually seen in younger patients
 TEN: usually patients above 60 years
 Initially present with flu-like symptoms
 After a few days, cutaneous lesions appear on trunk: Erythematous macules with Sloughing of the skin, flaccid bullae
 May have mucosal changes as wel

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31
Q

tx SJS/TEN reatment:
 Identify?
 Management in?
 Mortality rates

A

 Identify, immediately discontinue offending drug
 Management in burn unit of hospital
 Mortality rate: SJS – 1-5% , TEN – 25-30%

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32
Q
33
Q

Pemphigus vulgaris
 dx type?
 target?
 cases per million people

A

 Autoimmune disease
 Immune attack on desmosomes – intra-epithelial split (pemphigus)
 1-5 cases per million people

34
Q

Ag of pemphigus vulgaris

A

desmoglein 3

35
Q

Pemphigus vulgaris
 Clinical features:
 Oral lesions often?
 Avg diagnosis age:
 lesion app?
 Affect what sites?
 gingiva?
 Skin lesions?
 Ocular lesions?

A

 Oral lesions often first sign of disease, and most difficult to treat
 Avg diagnosis age: 50 years
 superficial, ragged erosions/ ulcerations
 Affect any oral mucosal location
 Desquamative gingivitis
 Skin lesions: flaccid vesicles and bullae that rupture quickly
 Ocular lesions: conjunctivitis

36
Q

pemphigus vulgaris
 Clinical features:
 Lesions without tx?
 pain?
 Skin: positive sign?

A

 Lesions persist and progress without treatment
 Lesions are painful
 Skin: positive Nikolsky sign: bulla appears with firm lateral pressure

37
Q

Pemphigus vulgaris
 Diagnosis:

A

 Clinical, histopathology, direct immunofluorescence
 10% Formalin vs Michels solution (2 biopsies, 1 for each soultion= DIF and histopath)
 Perilesional biopsy

38
Q

tx pemphigus vulgaris

A

Systemic corticosteroids, immunosuppressive drugs

39
Q

dif ?

A

erosive lichen planus
pemphigus vulgaris
mucus membrane pemphigoid

40
Q

dif?

A

LP
pemphigus vulgairis
erythema multiforme
mucus mem pemphigoid

41
Q

DIF reveals a autoAb to desmoglein 3

A

pemphigus vulgaris

42
Q

most likely dx?

A

pemphigus vulgaris

43
Q

firm lat pressure produced this

A

+ nikolsky, PV

44
Q

pemphigus vulgaris histo

A

unzipped epithelium, but basal layer still intact= tombstones

45
Q
A

pemphigus vulgaris, basal layer still present

46
Q

PV DIF

A

chickenwire pattern intraepi, Ab against desmoglein 3

47
Q

desmoglein 3

48
Q

Mucus membrane pemphigoid (Cicatricial pemphigoid)
 dx type?
 def?
 commonality vs pemphigus?
 ab?

A

 Autoimmune disease
 Chronic, blistering, mucocutaneous disease
 At least 2x more common compared to pemphigus
 Tissue-bound autoantibodies against components of basement membrane (ie:hemidesmosomes)

49
Q

mucus membrane pemphigoid clinical features
 age?
 MC mucosal site?
 Other sites?
 Oral lesions app?
 Lesions may?
 symptoms?

A

 50-60 years of age
 MC mucosal site: oral cavity
 Ocular, nasal, esophageal, laryngeal, vaginal mucosa
 Oral lesions: vesicles or bullae
 May rupture: large erosions and ulcerations
 Lesions are painful

50
Q

Mucus membrane pemphigoid (Cicatricial pemphigoid)
 Clinical features:
 May be observed in any?
 gingiva?
 Most significant complication? may result in?

A

 May be observed in any intraoral site
 Desquamative gingivitis
 Most significant complication: ocular involvement – symblepharon formation, May result in blindness

51
Q

Mucus membrane pemphigoid (Cicatricial pemphigoid)
 Diagnosis:

A

 Clinical, histopathology, direct immunofluorescence
 10% Formalin vs Michels solution
 Perilesional biopsy

52
Q

MMP tx
 If only oral lesions present?
 Patient should be referred to?
 OHI measures?

A

 If only oral lesions present, may be controlled with topical corticosteroids
 Patient should be referred to ophthalmologist
 OHI measures for gingival lesions

53
Q

dif?

A

erosive lichen planus
pemphigus vulgaris
mucus membrane pemphigoid

54
Q

60 y/o female, presents with ocular conjunctivitis as well

A

mucus membrane pemphigoid

55
Q

pt also has desquamtative gingivitis, what is most liklely?

A

mucus membrane pemphigoid

56
Q

MMP histo

A

similar to pemphigus but basal layer not intact, complete epithelial seperation from the BMZ
DIF reveal IgA and IgG in BMZ in linear form

57
Q

histo

A

mucus membrane pemphigoid

58
Q

IgG+, IgA+

59
Q

Bullous pemphigoid
 dx type?
 def?
 Ab?

A

 Autoimmune disease
 Chronic, blistering, mucocutaneous disease
 Tissue-bound autoantibodies against components of basement membrane (ie: hemidesmosomes)

60
Q

Bullous pemphigoid
 Clinical features:
 ages?
 early symptom?
 Bullae develop on? then?
 Healing?

A

 75-80 years
 Pruritis is often an early symptom
 Bullae develop on skin – rupture after several days
 Healing without scarring

61
Q

80 y/o female, only lesions like this on skin, likely dx?

A

bullous pemphigoid

62
Q

Systemic sclerosis/ Scleroderma
 May be?
 tissue depositions?
 what organs affected?

A

 May be immune-mediated condition
 Dense collagen deposited throughout the tissue
 Most organs of the body affected

63
Q

Systemic sclerosis
 Clinical features:
 gender bias?
 Mainly observed in what ages?
 Often first noticed by?
 Skin develops? Surface texture?

A

Systemic sclerosis
 Clinical features:
 Females: 2-3x more common
 Mainly observed in adults
 Often first noticed by cutaneous changes
 Skin develops diffuse, hard texture
 Surface is usually smooth

64
Q

Systemic sclerosis Clinical features:
 Involvement of facial skin?
 what develops with perioral involvement?
 Tongue?
 salivary glands?
 Nasal ala?
 often 1st sign of disease?
 phalanges?

A

 Involvement of facial skin: smooth, taut, mask-like appearance
 Microstomia develops with perioral involvement
 Tongue becomes stiff, dysphagia may develop
 Xerostomia may be present
 Nasal ala becomes atrophied – pinched appearance
 Raynaud phenomenon: vasoconstrictive event triggered by exposure to cold or stress
– often 1st sign of disease (fingers and toes)
 Resorption of terminal phalanges → shortened clawlike fingers

65
Q

Systemic sclerosis Clinical features:
 Involvement of other organs:
 May eventually lead to?
 example path?

A

 Involvement of other organs: fibrosis of lungs, heart, GI tract
 May eventually lead to organ failure
 Pulmonary fibrosis → pulmonary hypertension → heart failure

66
Q

Systemic sclerosis
 Radiographic features of the jaw:

A

 Widening of the PDL
 Resorption of posterior mandibular ramus, condyle, coronoid process

67
Q

Systemic sclerosis tx

A

prognosis is poor
 Systemic medications: penicillamine

68
Q

CREST syndrome

A

Calcinosis cutis, Raynaud phenomenon, Esophageal dysfunction, Sclerodactyly, Teliangiectasia

69
Q

CREST syndrome/ Limited scleroderma
 Clinical features:
 sex/age?
 Calcinosis cutis:
 Raynaud phenomenon:
 Esophageal dysfunction:
 Sclerodactyly:
 Telangiectasia:

A

 MC female, 6th – 7th decade
 Calcinosis cutis: movable, subcutaneous nodules
 Raynaud phenomenon: severe vasospasm in fingers/toes
 Esophageal dysfunction: abnormal collagen deposition
 Sclerodactyly: finger becomes stiff, skin – smooth, shiny appearance
 Telangiectasia: superficial dilated capillarie

71
Q
A

raynaud phenomenom

72
Q
A

SS terminal phalange resorb

73
Q

histo of systemic sclerosis

A

dense collagen in conn tissue layer

74
Q

what is likely

A

systemic sclerosis

75
Q

Crohn’s disease
 def?
 Anywhere in?
 Genetics?
 Oral lesions ?

A

 Inflammatory bowel disease, immune related
 Anywhere in GI tract – mouth to anus
 Genetic factor implicated
 Oral lesions may precede GI lesions

76
Q

Crohn’s disease
 Clinical features:
 MC Dx in?
 signs/symptoms?
 Oral? app?

A

 MC Dx in 2nd decade
 Abdominal cramping, pain, nausea, diarrhea
 Weight loss and malnutrition may develop → anemia, decreased growth
 Oral: diffuse, nodular swelling, ulcers: Cobblestone appearance, Erythematous macules and plaques

77
Q

Crohn’s disease
 Treatment:
 Oral lesions typically clear with?

A

 Oral lesions typically clear with GI treatment
 Sulfasalazine, antibiotics, corticosteroids

78
Q

23 y/o female, complains of cramps as well

A

Chron’s dx likely

79
Q

pt presents recent weight loss

PATH II (32 decks)

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