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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
erythema multiforme
26
erythema multiforme
27
skin lesions present as well with concentric rings
erythema multiforme
28
skin lesions with ringed app present as well
erythema multiforme
29
Stevens – Johnson syndrome and Toxic epidermal necrolysis
 Severe blistering diseases triggered by drug exposure  SJS: <10% skin and mucosal involvement  TEN: >30% skin and mucosal involvement
30
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?
 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
31
tx SJS/TEN reatment:  Identify?  Management in?  Mortality rates
 Identify, immediately discontinue offending drug  Management in burn unit of hospital  Mortality rate: SJS – 1-5% , TEN – 25-30%
32
sjs/ten
33
Pemphigus vulgaris  dx type?  target?  cases per million people
 Autoimmune disease  Immune attack on desmosomes -- **intra-epithelial** split (pemphigus)  1-5 cases per million people
34
Ag of pemphigus vulgaris
desmoglein 3
35
Pemphigus vulgaris  Clinical features:  Oral lesions often?  Avg diagnosis age:  lesion app?  Affect what sites?  gingiva?  Skin lesions?  Ocular lesions?
 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
pemphigus vulgaris  Clinical features:  Lesions without tx?  pain?  Skin: positive sign?
 Lesions persist and progress without treatment  Lesions are painful  Skin: positive Nikolsky sign: bulla appears with firm lateral pressure
37
Pemphigus vulgaris  Diagnosis:
 Clinical, histopathology, direct immunofluorescence  10% Formalin vs Michels solution (2 biopsies, 1 for each soultion= DIF and histopath)  Perilesional biopsy
38
tx pemphigus vulgaris
Systemic corticosteroids, immunosuppressive drugs
39
dif ?
erosive lichen planus pemphigus vulgaris mucus membrane pemphigoid
40
dif?
LP pemphigus vulgairis erythema multiforme mucus mem pemphigoid
41
DIF reveals a autoAb to desmoglein 3
pemphigus vulgaris
42
most likely dx?
pemphigus vulgaris
43
firm lat pressure produced this
+ nikolsky, PV
44
pemphigus vulgaris histo
unzipped epithelium, but basal layer still intact= tombstones
45
pemphigus vulgaris, basal layer still present
46
PV DIF
chickenwire pattern intraepi, Ab against desmoglein 3
47
desmoglein 3
PV
48
Mucus membrane pemphigoid (Cicatricial pemphigoid)  dx type?  def?  commonality vs pemphigus?  ab?
 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
mucus membrane pemphigoid clinical features  age?  MC mucosal site?  Other sites?  Oral lesions app?  Lesions may?  symptoms?
 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
Mucus membrane pemphigoid (Cicatricial pemphigoid)  Clinical features:  May be observed in any?  gingiva?  Most significant complication? may result in?
 May be observed in any intraoral site  Desquamative gingivitis  Most significant complication: ocular involvement – symblepharon formation, May result in blindness
51
Mucus membrane pemphigoid (Cicatricial pemphigoid)  Diagnosis:
 Clinical, histopathology, direct immunofluorescence  10% Formalin vs Michels solution  Perilesional biopsy
52
MMP tx  If only oral lesions present?  Patient should be referred to?  OHI measures?
 If only oral lesions present, may be controlled with topical corticosteroids  Patient should be referred to ophthalmologist  OHI measures for gingival lesions
53
dif?
erosive lichen planus pemphigus vulgaris mucus membrane pemphigoid
54
60 y/o female, presents with ocular conjunctivitis as well
mucus membrane pemphigoid
55
pt also has desquamtative gingivitis, what is most liklely?
mucus membrane pemphigoid
56
MMP histo
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
histo
mucus membrane pemphigoid
58
IgG+, IgA+
MMP
59
Bullous pemphigoid  dx type?  def?  Ab?
 Autoimmune disease  Chronic, blistering, mucocutaneous disease  Tissue-bound autoantibodies against components of basement membrane (ie: hemidesmosomes)
60
Bullous pemphigoid  Clinical features:  ages?  early symptom?  Bullae develop on? then?  Healing?
 75-80 years  Pruritis is often an early symptom  Bullae develop on skin – rupture after several days  Healing without scarring
61
80 y/o female, only lesions like this on skin, likely dx?
bullous pemphigoid
62
Systemic sclerosis/ Scleroderma  May be?  tissue depositions?  what organs affected?
 May be immune-mediated condition  Dense collagen deposited throughout the tissue  Most organs of the body affected
63
Systemic sclerosis  Clinical features:  gender bias?  Mainly observed in what ages?  Often first noticed by?  Skin develops? Surface texture?
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
Systemic sclerosis Clinical features:  Involvement of facial skin?  what develops with perioral involvement?  Tongue?  salivary glands?  Nasal ala?  often 1st sign of disease?  phalanges?
 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
Systemic sclerosis Clinical features:  Involvement of other organs:  May eventually lead to?  example path?
 Involvement of other organs: fibrosis of lungs, heart, GI tract  May eventually lead to organ failure  Pulmonary fibrosis → pulmonary hypertension → heart failure
66
Systemic sclerosis  Radiographic features of the jaw:
 Widening of the PDL  Resorption of posterior mandibular ramus, condyle, coronoid process
67
Systemic sclerosis tx
prognosis is poor  Systemic medications: penicillamine
68
CREST syndrome
 **C**alcinosis cutis, **R**aynaud phenomenon, **E**sophageal dysfunction, **S**clerodactyly, **T**eliangiectasia
69
CREST syndrome/ Limited scleroderma  Clinical features:  sex/age?  Calcinosis cutis:  Raynaud phenomenon:  Esophageal dysfunction:  Sclerodactyly:  Telangiectasia:
 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
70
SS
71
raynaud phenomenom
72
SS terminal phalange resorb
73
histo of systemic sclerosis
dense collagen in conn tissue layer
74
what is likely
systemic sclerosis
75
Crohn’s disease  def?  Anywhere in?  Genetics?  Oral lesions ?
 Inflammatory bowel disease, immune related  Anywhere in GI tract – mouth to anus  Genetic factor implicated  Oral lesions may precede GI lesions
76
Crohn’s disease  Clinical features:  MC Dx in?  signs/symptoms?  Oral? app?
 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
Crohn’s disease  Treatment:  Oral lesions typically clear with?
 Oral lesions typically clear with GI treatment  Sulfasalazine, antibiotics, corticosteroids
78
23 y/o female, complains of cramps as well
Chron's dx likely
79
pt presents recent weight loss
chrons

PATH II (32 decks)

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