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Pathology II > Skin and Mucosal Diseases II > Flashcards

Skin and Mucosal Diseases II Flashcards

1
Q

describe lichen planus

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

what are the clinical features of lichen planus

A
  • Most common in middle aged female adults
  • skin lesions: purple, pruritic, polygonal, papules (4-Ps)
  • often affect flexor surfaces of extremities
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3
Q

what are the clinical features of reticular lichen planus

A
  • most common
  • involves buccal mucosa bilaterally
  • interlacing white lines- Wickham striae
  • wax and wane
  • post inflammatory melanosis in darker skin patients
  • usually asymptomatic
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4
Q

what are the clinical features of erosive type lichen planus

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

what is the dx for lichen planus

A
  • clinical, histopathology, direct immunofluorescence
  • 10% formaling vs Michels solution
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6
Q

when do you use michels solution

A

when you need to see direct immunofluorescence

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7
Q

what is the tx for lichen planus

A
  • reticular: usually asymptomatic, no tx needed
  • erosive: topical corticosteroids
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8
Q

describe erythema multiforme

A
  • ulcerative mucocutaneous condition of uncertain etiology
  • likely immune mediated
  • 50% of cases: precipitating cause- infections such as herpes simplex, medications
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9
Q

what are the clinical features of erythema multiforme

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- bullseye
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10
Q

where are the lesions found in erythema multiforme

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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11
Q

what are the clinical features of erythema multiforme minor

A
  • milder cases
  • skin lesions and 1 mucosal site usually oral
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12
Q

what are the clinical features of erythema multiforme major

A
  • most severe
  • widespread skin lesions and 2 or more mucosal sites
  • severe ocular involvement: scarring (symblepharon formation)
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13
Q

what is the tx for erythema multiforme

A
  • usually self-limiting (2-6 weeks)
  • systemic or topical corticosteroids
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14
Q

what is Stevens-Johnson syndrome and toxic epidermal necrolysis

A

severe blistering diseases triggered by drug exposure

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15
Q

what is the skin and mucosal involvement in SJS and TEN

A
  • SJS: less than 10% skin and mucosal involvement
  • TEN: greater than 30% skin and mucosal involvement
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16
Q

what are the clinical features of SJS and TEN

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, sloughing of the skin, flaccid bullae
  • may have mucosal changes as well
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17
Q

what is the tx for SJS and TEN

A
  • identify, immediately discontinue offending drug
  • management in burn unit of hospital
  • mortality rate : SJS is 1-5% and TEN is 25-30%
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18
Q

describe pemphigus vulgaris

A
  • autoimmune disease
  • immune attack on desmosomes- intra epithelial split
  • 1-5 cases per million people
  • blistering
  • antibodies against desmosomes that hold epithelial cells together
19
Q

what are the clinical features of pemphigus vulgaris

A
  • oral lesions often first sign of disease and most difficult to treat
  • average diagnosis 50 years
  • superficial, ragged erosions and ulcerations
  • affect any oral mucosal location
  • desquamative gingivitis
  • skin lesions: flaccid vesicles and bullae that rupture quickly
  • ocular lesions: conjunctivitis
20
Q

what are the clinica features of pemphigus vulgaris

A
  • lesions persist and progress without treatment
  • lesions are painful
  • skin: positive Nikolsky sign: bulla appears with firm lateral pressure
21
Q

what is the dx with pemphigus vulgaris

A
  • clinical, histopathology, direct immunofluorescence
  • 10% formalin vs michels solution
  • perilesional biopsy
22
Q

what is the tx for pemphigus vulgaris

A
  • systemic corticosteroids
  • immunosuppressive drugs
23
Q

describe mucus membrane pemphigoid

A
  • autoimmune disease
  • chronic, blistering, mucocutaneous disease
  • at least 2x more common compared to pemphigus
  • tissue- bound autoantibodies against components of basement membrane - hemidesmosomes
24
Q

what are the clinical features of mucus membrane pemphigoid

A
  • 50-60 years of age
  • most common mucosal site: oral cavity
  • ocular, nasal, esophageal, laryngeal, vaginal mucosa
  • oral lesions: vesicles or bullae
  • may rupture: large erosions and ulcerations
  • lesions are painful
  • may be observed in any intraoral site
  • desquamative gingivitis
25
Q

what is the most significant complication in mucus membrane pemphigoid

A
  • ocular involvement - symblepharon formation
  • may result in blindness
26
Q

what is another name for mucus membrane pemphigoid

A

cicatricial pemphigoid

27
Q

what is the dx for mucus membrane pemphigoid

A
  • clinical, histopathology, direct immunofluorescence
  • 10% formalin vs michels solution
  • perilesional biopsy
28
Q

what is the tx for mucus membrane pemphigoid

A
  • if only oral lesions are present, may be controlled with topical corticosteroids
  • patient should be refeerred to opthamologist
  • OHI measures can improve gingival lesions
29
Q

describe bullous pemphigoid

A
  • autoimmune disease
  • chronic, blistering, mucocutaneous disease
  • tissue- bound autoantibodies against components of basement membrane - hemidesmosomes
30
Q

what are the clinical features of bullous pemphigoid

A
  • 75-80 years
  • pruritis is often an early symptom
  • bullae develop on skin- rupture after several days
  • healing without scarring
31
Q

describe systemic sclerosis

A
  • may be immune mediated condition
  • dense collagen deposited throughout the tissue
  • most organs of the body affected
32
Q

what are the clinical features of systemic sclerosis

A
  • females: 2-3x more common
  • mainly observed in adults
  • often first noticed by cutaneous changes
  • skin develops diffuse, hard texture
  • surface is usually smooth
  • involvement of facial skin: smooth, taut, mask-like apperance
  • microstomia
  • tongue becomes stiff, dysphagia may develop
  • xerostomia
  • nasal ala decomes atrophied- pinched appearance
  • raynaud phenomenon
  • resorption of terminal phalanges -> shortened clawlike fingers
33
Q

describe raynaud phenomenon

A

vasoconstrictive event triggered by exposure to cold or stress- often the 1st sign of disease

34
Q

what organs are involed in systemic sclerosis

A
  • fibrosis of lungs, heart, GI tract
  • may eventually lead to organ faulure
  • pulmonary fibrosis -> pulmonary hypertension -> HF
35
Q

what are the radiographic features of systemic sclerosis

A
  • widening of PDL
  • resorption of posterior mandibular ramus, condyle, coronoid process
36
Q

what is the tx for systemic sclerosis

A
  • prognosis is poor
  • systemic medications: penicillamine
37
Q

what are the components of crest syndrome

A
  • calcinosis cutis
  • raynaud phenomenon
  • esophageal dysfunction
  • sclerodactyly
  • telliangiectasia
38
Q

what is another name for CREST syndrome

A

limited scleroderma

39
Q

what are the clinical featurs of CREST synromr

A
  • most common in females 6-7th decade of life
  • calcinosis cutis: movable, subcutaneous ndules
  • raynaud phenomenon: severe vasopasm in fingers/toes
  • esophageal dysfunction: abnormal collagen deposition
  • sclerodactyly: fingers become stiff, skin - smooth, shiny appearance
  • telangiectasia: superficial dilated capillaries
40
Q

describe Crohn’s disease

A
  • inflammatory bowel disease, immune related
  • anywhere in GI tract- mouth to anus
  • genetic factor implicated
  • oral lesions may precede GI lesions
41
Q

what are the clinical feautres of Crohn’s disease

A
  • most common 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
42
Q

what is the tx for crohn’s disease

A
  • oral lesions tpyically clear with GI tx
  • sulfasalazine, antibiotics, corticosteroids
43
Q
A

Pathology II (19 decks)

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