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xD2 Oral Path Final Exam > OPF Epithelial Disorders (Ch16) > Flashcards

OPF Epithelial Disorders (Ch16) Flashcards

1
Q

What is a group of inherited conditions in which two or more ectodermally derived anatomic structures fail to develop?

A

Ectodermal dysplasia

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2
Q

Ectodermal dysplasia can affect what?

A 
Skin 
Hair 
Nails 
Teeth 
Sweat glands
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3
Q

What did Dr Svirsky emphasize as the three things that ectodermal dysplasia do?

A
  1. Sparse sweat
  2. Sparse hair
  3. Hypodontia
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4
Q

How is ectodermal dysplasia inherited?

A

Autosomal dominant

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5
Q

What are skin and hair problems associated with Ectodermal dysplasia?

A

Sparse hair, eyebrows (blond)
Periocular hyperpigmentation (dark circles around eyes)
Dystrophic nails

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6
Q

Why would an ectodermal dysplasia patient have xerostomia?

A

Decreased salivary gland development

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7
Q

What are Tooth problems with ectodermal dysplasia?

A

Hypodontia

Cone shaped teeth

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8
Q

What are 2 treatments for Ectodermal dysplasia?

A

Genetic counseling (w/ Dr. Elsea)
Prosthetic teeth
Do a pedigree and a microarray

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9
Q

What is autosomal dominant inherited skin disorder manifesting as thick white buccal mucosa bilaterally, may be corrugated or velvety

A

White sponge nevus

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10
Q

Is White Sponge nevus malignant and is there treatment?

A

Benign; no treatment

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11
Q

What is an autosomal dominant condition seen in descendants of North Carolina Indians, with thick white plaques on the buccal mucosa(like white sponge nevus) and the conjunctiva?

A

Hereditary Benign Intraepithelial Dyskeratosis

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12
Q

Histology Dr Siversky said to know for Hereditary Benign Intraepithelial Dyskeratosis?

A

Epithelial clefting

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13
Q

What is an autosomal dominant skin problem with erythematous puritic papules on the trunk and scalp with foul odor, dystrophic nails (ridged and split)?

A

Keratosis follicularis (Darier’s disease)

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14
Q

What is the oral manifestations of Keratosis Follicularis/ Darier’s Disease?

A

Multiple white papules

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15
Q

How do you treat Keratosis Follicularis/ Darier’s Disease?

A

Keratolytic agents or emollients

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16
Q

What is histopathologically identical to Darier’s Disease , but manifests orally as a single white papule?

A

Warty dyskeratosis

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17
Q

Where does the single white papule of Warty dsykeratoma appear?

A

on hard palate or alveolar ridge

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18
Q

What is an autosomal dominant with freckle like lesions in and around oral cavity, on the hands, and having intestinal polyps with a predisposition to go to adenocarcinoma?

A

Peutz-Jeghers

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19
Q

What is the difference, as far as the intestinal polyps, between Peutz-Jeghers and Gardners Syndrome?

A

Peutz-Jeghers has benign intestinal polyps while Garderns are going to go malignant

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20
Q

What is the treatment for Peutz-Jeghers?

A

Genetic counseling Monitor for intussusceptions(one part of intestine invaginates into another section, like a telescope [Wikipedia] or malignant transformation

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21
Q

What is an autosomal dominant conditions of multiple vascular hamartomas due to decreased blood vessel wall intregrity. Pt can have frequent epistaxis (nose bleeds) . Telangiectasias can be intraoral, hands, feet, GI tract, GU tract and eye?

A

Hereditary Hemorrhagic telangiectasia

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22
Q

With any bleeding disorder, even Hereditary Hemoorhagic Telangiectasia, what should always be considered as another problem that might follow?

A

Iron deficiency anemia

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23
Q

Genetic abnormalities that lead to abnormal collagen manifesting as hypermobile joints, elasticity of skin (carnival person)

A

Ehlers-Danlos Syndrome

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24
Q

What are some oral concerns with Ehlers-Danlos?

A

Subluxation of TMJ
Don’t respond well to surgery due to defective collagen
Bruise and bleed easily

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25
Q

What is a chronic inflammatory disease causing white striations, papules or plaques on buccal mucosa, tongue and gingival, thought to be autoimmune related by CD8 T cells causing oral epithelial cells apoptosis?

A

Lichen planus

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26
Q

What are the demographics for Lichen planus?

A

45-60 y.o (rare in children) Women 2x more than men

Skin lesions are much less common than intraoral

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27
Q

Is Lichen planus only caused by autoimmune response?

A

No, there can be drugs whose side effects cause a Lichenoid drug reaction, however, that goes away when drugs are stopped. True Lichen planus is chronic

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28
Q

If you see this phrase, you should think of Lichen Planus

A

Striae of Wickham

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29
Q

What is the histology for Lichen Planus?

A

Saw tooth rete pegs and loose basal cell layer

Pink layer of lymphocytes

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30
Q

What are people with persistent mouth sores at an increased risk for?

A

Squamous cell carcinoma

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31
Q

When biopsying Lichen planus, do you biopsy the red or the white area?

A

White area. Red is ulceration and will only show that.

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32
Q

What are the SKIN manifestations of Lichen planus?

A

Purple puritic polygonal papules

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33
Q

Which phase of Lichen planus is treated: Reticular or erosive, and with what?

A

Erosive. Corticosteroids because it is autoimmune mediated

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34
Q

What can superimpose on top of Lichen planus especially during corticosteroid treatment of the Erosive form ?

A

Candidiasis

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35
Q

What are some causes for lichen planus?

A

Allergic or immunologic reaction
20% medication induced
Linked to chronic hepatic diseases especially Hepatitis C
Psycogenic (stress and anxiety)
Associated with LP of skin, HBP and diabetes
Dental materials
** Cause may be T-cell mediated but actual cause is unknown

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36
Q

What is the connection with GVHD and Lichen Planus?

A

LP can be considered a form of GVHD that takes place in an individual who has received a bone marrow transplant.

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37
Q

Is there a relation between Lichen Planus and hepatitis C?

A

Yes but it might be a reaction to the drugs used to treat Hep C

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38
Q

What percent of patients with oral lichen planus have skin lesions?

A

5%

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39
Q

What percent of women and men have genital involvement of Lichen Planus?

A

25% of women

2-4% of men

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40
Q

Can nails and conjunctive be affected by lichen planus?

A

Yes but it is very rare

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41
Q

What are the key aspects of monitoring lichen planus?

A 
Educate patients
Photograph
Erosive form see four times a year
Biopsy
Reassure
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42
Q

What is a dislodgement of skin by lateral pressure, can be a symptom in multiple diseases?

A

Nikolsky Sign

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43
Q

What 2 intraroal diseases have a positive Nikolsky sign?

A
  1. Pemphigus

2. Pemphigoid

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44
Q

What does BMMP stand for?

A

Benign mucous membrane pemphigoid

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45
Q

Histologically, which has a change in the basement membrane: Pemphigoid or pemphigous?

A

Goid (goid goes down to basement)

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46
Q

What is the most pressing problem associated with BMMP?

A

Oral Lesions (conjunctival, nasal, esophageal, laryngeal & vaginal)

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47
Q

Pemphigoid type that affects women over the age of 60 manifesting orally as vesicles or bullae?

A

Benign mucous membrane pemphigoid (BMMP)

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48
Q

What is an entropian?

A

Scarring/eyelids turn inward

49
Q

What is trichiasis?

A

Eyelashes rub cornea and globe and scarring closes openings of lacrimal glands)

50
Q

What are symblepharons?

A

Adhesions

51
Q

What three ocular problems occur in 25% of BMMP patients?

A
  1. Symblepharons
  2. Entropion
  3. trichiasis
52
Q

What ocular findings are in BMMP (other than the three on another flashcard)?

A

With loss of tears, the eye becomes extremely dry
Cornea produces protective keratin
Keratin opaque and blindness ensues
Adhesions between upper and lower eyelid

53
Q

What is the histology for benign mucous membrane pemphigoid?

A
  1. Subepithelial clefting

2. Immunofluorescence at basement membrane

54
Q

How do you treat BMMP?

A 
Dapsone (cannot be G6PD deficient)
Steroids
Tetracycline/doxycycline 
Nicotinomide
Immunosuppresive agents
55
Q

The following are clinical characteristics of what pathology?

  • Vesicles, bullae and ulcers occur anywhere in oral cavity
  • Blood filled characteristic
  • Complaints of oral bleeding, sore gums and difficulty in swallowing
A

BMMP

56
Q

If going to treat someone with benign mucous membrane pemphigoid with Dapsone, what must you check first?

A

that they are not Glucose-6-Phosphate dehydrogenase deficient. Can cause aplastic anemia

57
Q

What is a mucocutaneous autoimmune disease that has intrapepithelial acantholysis resulting in intraepithelial clefting and is fatal If untreated?

A

Pemphigus vulgaris

58
Q

What is usually the first sign of Pemphigus Vulgaris, but also the hardest to treat?

A

Oral lesion, “first to show, last to go”

59
Q

Pemphigus Vulgaris intraepithelial clefting has what in the middle?

A

Tzank cells (floating cells in the intraepithelial cleft)

60
Q

The intraepithelial separation just above the basement membrane in Pemphigus casues what look histologically

A

Row of tombstones (the basal layer sticking up into the intraepithelial cleft)

61
Q

What is a major distinction demographically between pemphigus and pemphigoid?

A

Pemphigous affects M=F
Pemphigoid prefers males
Pemphigous has Jewish predilection

62
Q

How can you tell if a lesion is intra or sub-epithelial?

A

Good question. You need to find out.

63
Q

The following are clinical manifestations of what pathology?

  • Oral lesions precede skin
  • Large ragged ulcerations
  • Marginal gingival erosions early
A

Pemphigus vulgaris

64
Q

What causes the blistering in pemphigus?

A

autoimmune attack of desmosomes

65
Q

What is a pemphigus type caused by cross-reacting antibodies in lymphoma or leukemia attacking desmosomal complexes that may precede the discovery of an underlying malignancy?

A

paraneoplastic pemphigus/ neoplasia-induced pemphigus

66
Q

What pathology usually occurs in recipients of allogenic bone marrow transplants?

A

Graft Versus Host Disease

67
Q

What is the only transplant that does not require immunosuppressive drugs for life?

A

successful bone marrow transplant

68
Q

What factors can make Graft versus host disease milder?

A

Young pt
Good histocompatability match
Cord blood
Female

69
Q

What form of Graft versus host disease seen within a few weeks of transplant and can manifest as a mild rash to severe sloughing that resembles Toxic epidermal necrolysis?

A

Acute Graft Versus Host Disease

70
Q

What form of can be seen 100 days to years after transplant. Considered a continuation of acute graft versus host disease. Will mimic an autoimmune disease?

A

Chronic Graft Versus Host Disease

71
Q

Chronic Graft Versus Host disease will resemble what clinically?

A

Lichen planus or systemic sclerosis

72
Q

Which manifests more orally, Chronic or Acute Graft versus host disease

A

Chronic (80%)

73
Q

Beyond ulcers, what are 2 other oral manifestations of oral Graft Versus Host Disease?

A

Xerostomia due to immunologica destruction of salivary glands Mucocelles on soft palate (not as common)

74
Q

Mucoceles, which are seen commonly on lips and buccal mucosa of young males, if seen on the soft palat, one should think what?

A

Graft vs host disease

75
Q

If the ulcers last longer than 2 weeks beyond the 2 week post bone marrow transplant buffer zone have what?

A

Acute graft vs host disease

76
Q

What is the Primary strategy for dealing with Graft versus host disease?

A

reduce or prevent occurrence with good histocompatability match

77
Q

Common chronic skin disease characterized by an increased proliferative activity of the cutaneous keratinocytes. Mild disease that can be treated with keratolytic agents, sunlight, Vit D analogues

A

Psoriasis

78
Q

What is THE classic and one of the most common example of an immunologically mediated condition?

A

Lupus Erythematosus

79
Q

What is the most common of the collagen vascular and connective tissue disease in the US

A

Lupus Erythematosus

80
Q

Do we need to know the histology of psoriasis?

A

Nope

81
Q

Most immunologic diseases, including Lupus Erythematous, affect which gender predominantly?

A

Female (8 times more likely)

82
Q

Systemic lupus is caused by increased activity of what part of the immune system?

A

humoral limb (B lymphocytes [stimulated by abnormally functioning T lymphocytes ?])

83
Q

What are common findings of systemic lupus erythematosus?

A 
Fever 
Weight loss 
Arthritis 
Fatigue 
Malaise 
Characteristic butterfly pattern rash over malar area and nose that spares the nasolabial folds and is exacerbated by sun exposure
84
Q

What is the most significant aspect of systemic lupus erythematosus?

A

Kidney failure

85
Q

What is another systemic mortality risk in systemic lupus erythematosus?

A

bacterial endocarditis

86
Q

How can systemic lupus erythematous manifest orally?

A

Look lichenoid on palatal, buccal mucosa, and gingival. Ulcers, pain, erythema, hyperkeratosis

87
Q

if Lupus manifests on the vermillion zone of the lower lip it is called what?

A

Lupus cheilitis

88
Q

What is a Lupus type with no systemic symptoms, and only lesions on the skin and mucosal surfaces, called discoid lupus erythematous and present on sun-exposed areas of skin?

A

Chronic cutaneous Lupus erythematosus

89
Q

What is a lab test that is high in Lupus erythematous patients?

A

high in ANA = autonucleic antibody (higher than in any other disease)

90
Q

What is the most common cause of death in systemic lupus erythematosus?

A

Renal failure

91
Q

What are treatments for Lupus if mild?

A

Avoid sun
NSAIDs
Antimalarials (hydroxychloroquine)

92
Q

What is the treatment for Lupus if severe?

A

corticosteroids and other immunosuppressives

93
Q

If you see lichen planus, what else can be put on the differential?

A

Lupus erythematous

94
Q

What is an mmunologically mediated deposition of dense collagen in tissues also known as Scleroderma?

A

Systemic sclerosis

95
Q

What is the first sign of systemic sclerosis, but is not diagnostic for the disease?

A

Raynaud’s phenomenon

96
Q

Systemic sclerosis higher in which gender?

A

Females

97
Q

What is the cause of death in systemic sclerosis?

A

pulmonary fibrosis, leading to pulmonary hypertension and heart failure

98
Q

What does pulmonary fibrosis do?

A

reduces lung surface area for oxygen exchange

99
Q

What is the clinical appearance of a person with systemic sclerosis?

A

smooth, taut, mask-like face, nasal alae atrophy = mouse facies

100
Q

What is the character of the mouth of pt with systemic sclerosis?

A

microstomia with purse-string furrows radiating from the mouth

101
Q

What is the character of the gingiva in pt with systemic sclerosis?

A

Lose attached gingiva

102
Q

What are symptoms of oral systemic sclerosis?

A

Widened periodontal ligament space
Resorption of posterior ramus, coronoid process and condyle in 10-15% of patients (resorbed due to increased pressure associated with abnormal collagen production)

103
Q

What is resorption of terminal phalnages and flexure contractures producing club like fingers in systemic sclerosis and some other diseases?

A

Acro-osteolysis

104
Q

Does systemic sclerosis with its widened PDL and loss of attached gingival respond to periodontal therapy?

A

No

105
Q

Siversky loves this disease and brought up the fact that this disease, radiographically, shows a loss of the lamina dura

A

Hyperparathyroidism

106
Q

What is a systemic sclerosis that is localized usually leaving a scar called en coup de sabre, Dr, Siversky mentioned it as Morpha?

A

localized scleraderma

107
Q

What can the pressure of collagen deposition cause orally and skeletally?

A

resorption of posterior ramus, coronoid process and condyle

108
Q

What systemic medication is prescribed to try and inhibit collagen production in systemic sclerosis?

A

D-penicillamine

109
Q

Prescribing calcium channel blockers to increase peripheral blood flow or Angiotensin-converting enzyme inhibitors if the kidney is involved, all have the goal of doing what for systemic sclerosis?

A

slow the disease process

110
Q

would it be smart to fabricate dentures for a systemic sclerosis patient?

A

No due to microstomia and inelasticity of the mouth

111
Q

What does CREST stand for?

A

Calcinosis cutis Raynaud’s phenomenon Esophageal dysfunction Sclerodactyly Telangiectasias

112
Q

What part of CREST syndrome is characterized by movable, non-tender, subcutaneous nodules, .5cm to 2cm in size caused by the deposition of calcium salts?

A

Calcinosis cutis

113
Q

What is Part of CREST as well as other syndromes characterized by dramatic blanching of digits (dead white) when exposed to cold or even stress. Digit will turn blue a few minutes later with venous stasis, then a final dusky red hue as hyperemic blood flow returns. May be accompanied by throbbing pain?

A

Ranaud’s phenomenon

114
Q

Is Raynaud’s phenomenon indicative of scleraderma or CREST?

A

No

115
Q

Diagnosed with a barium swallow, this part of CREST is due to abnormal collagen deposition in esophageal submucosa. It is not noticed early in CREST, but will affect swallowing later.

A

Esophageal dysfunction

116
Q

What part of CREST is caused by abnormal deposition of collagen in the dermis leading to fingers becoming stiff, skin becoming shiny, and digits can undergo permanent flexture to a claw-like deformity?

A

Sclerodactyly

117
Q

What part of CREST due to fragile vessels, and is similar to Hereditary Hemorrhagic Telangiectasia?

A

Telangiectasias

118
Q

Which has better prognosis, CREST or systemic sclerosis/scleraderma?

A

CREST

xD2 Oral Path Final Exam (6 decks)

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