OPF Epithelial Disorders (Ch16) Flashcards
(118 cards)
1
Q
What is a group of inherited conditions in which two or more ectodermally derived anatomic structures fail to develop?
A
Ectodermal dysplasia
2
Q
Ectodermal dysplasia can affect what?
A
Skin Hair Nails Teeth Sweat glands
3
Q
What did Dr Svirsky emphasize as the three things that ectodermal dysplasia do?
A
- Sparse sweat
- Sparse hair
- Hypodontia
4
Q
How is ectodermal dysplasia inherited?
A
Autosomal dominant
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
6
Q
Why would an ectodermal dysplasia patient have xerostomia?
A
Decreased salivary gland development
7
Q
What are Tooth problems with ectodermal dysplasia?
A
Hypodontia
Cone shaped teeth
8
Q
What are 2 treatments for Ectodermal dysplasia?
A
Genetic counseling (w/ Dr. Elsea)
Prosthetic teeth
Do a pedigree and a microarray
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
10
Q
Is White Sponge nevus malignant and is there treatment?
A
Benign; no treatment
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
12
Q
Histology Dr Siversky said to know for Hereditary Benign Intraepithelial Dyskeratosis?
A
Epithelial clefting
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)
14
Q
What is the oral manifestations of Keratosis Follicularis/ Darier’s Disease?
A
Multiple white papules
15
Q
How do you treat Keratosis Follicularis/ Darier’s Disease?
A
Keratolytic agents or emollients
16
Q
What is histopathologically identical to Darier’s Disease , but manifests orally as a single white papule?
A
Warty dyskeratosis
17
Q
Where does the single white papule of Warty dsykeratoma appear?
A
on hard palate or alveolar ridge
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
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
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
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
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
23
Q
Genetic abnormalities that lead to abnormal collagen manifesting as hypermobile joints, elasticity of skin (carnival person)
A
Ehlers-Danlos Syndrome
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
25
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?
Lichen planus
26
What are the demographics for Lichen planus?
45-60 y.o (rare in children) Women 2x more than men
| Skin lesions are much less common than intraoral
27
Is Lichen planus only caused by autoimmune response?
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
28
If you see this phrase, you should think of Lichen Planus
Striae of Wickham
29
What is the histology for Lichen Planus?
Saw tooth rete pegs and loose basal cell layer
| Pink layer of lymphocytes
30
What are people with persistent mouth sores at an increased risk for?
Squamous cell carcinoma
31
When biopsying Lichen planus, do you biopsy the red or the white area?
White area. Red is ulceration and will only show that.
32
What are the SKIN manifestations of Lichen planus?
Purple puritic polygonal papules
33
Which phase of Lichen planus is treated: Reticular or erosive, and with what?
Erosive. Corticosteroids because it is autoimmune mediated
34
What can superimpose on top of Lichen planus especially during corticosteroid treatment of the Erosive form ?
Candidiasis
35
What are some causes for lichen planus?
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
36
What is the connection with GVHD and Lichen Planus?
LP can be considered a form of GVHD that takes place in an individual who has received a bone marrow transplant.
37
Is there a relation between Lichen Planus and hepatitis C?
Yes but it might be a reaction to the drugs used to treat Hep C
38
What percent of patients with oral lichen planus have skin lesions?
5%
39
What percent of women and men have genital involvement of Lichen Planus?
25% of women
| 2-4% of men
40
Can nails and conjunctive be affected by lichen planus?
Yes but it is very rare
41
What are the key aspects of monitoring lichen planus?
```
Educate patients
Photograph
Erosive form see four times a year
Biopsy
Reassure
```
42
What is a dislodgement of skin by lateral pressure, can be a symptom in multiple diseases?
Nikolsky Sign
43
What 2 intraroal diseases have a positive Nikolsky sign?
1. Pemphigus
| 2. Pemphigoid
44
What does BMMP stand for?
Benign mucous membrane pemphigoid
45
Histologically, which has a change in the basement membrane: Pemphigoid or pemphigous?
Goid (goid goes down to basement)
46
What is the most pressing problem associated with BMMP?
Oral Lesions (conjunctival, nasal, esophageal, laryngeal & vaginal)
47
Pemphigoid type that affects women over the age of 60 manifesting orally as vesicles or bullae?
Benign mucous membrane pemphigoid (BMMP)
48
What is an entropian?
Scarring/eyelids turn inward
49
What is trichiasis?
Eyelashes rub cornea and globe and scarring closes openings of lacrimal glands)
50
What are symblepharons?
Adhesions
51
What three ocular problems occur in 25% of BMMP patients?
1. Symblepharons
2. Entropion
3. trichiasis
52
What ocular findings are in BMMP (other than the three on another flashcard)?
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
What is the histology for benign mucous membrane pemphigoid?
1. Subepithelial clefting
| 2. Immunofluorescence at basement membrane
54
How do you treat BMMP?
```
Dapsone (cannot be G6PD deficient)
Steroids
Tetracycline/doxycycline
Nicotinomide
Immunosuppresive agents
```
55
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
BMMP
56
If going to treat someone with benign mucous membrane pemphigoid with Dapsone, what must you check first?
that they are not Glucose-6-Phosphate dehydrogenase deficient. Can cause aplastic anemia
57
What is a mucocutaneous autoimmune disease that has intrapepithelial acantholysis resulting in intraepithelial clefting and is fatal If untreated?
Pemphigus vulgaris
58
What is usually the first sign of Pemphigus Vulgaris, but also the hardest to treat?
Oral lesion, “first to show, last to go”
59
Pemphigus Vulgaris intraepithelial clefting has what in the middle?
Tzank cells (floating cells in the intraepithelial cleft)
60
The intraepithelial separation just above the basement membrane in Pemphigus casues what look histologically
Row of tombstones (the basal layer sticking up into the intraepithelial cleft)
61
What is a major distinction demographically between pemphigus and pemphigoid?
Pemphigous affects M=F
Pemphigoid prefers males
Pemphigous has Jewish predilection
62
How can you tell if a lesion is intra or sub-epithelial?
Good question. You need to find out.
63
The following are clinical manifestations of what pathology?
- Oral lesions precede skin
- Large ragged ulcerations
- Marginal gingival erosions early
Pemphigus vulgaris
64
What causes the blistering in pemphigus?
autoimmune attack of desmosomes
65
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?
paraneoplastic pemphigus/ neoplasia-induced pemphigus
66
What pathology usually occurs in recipients of allogenic bone marrow transplants?
Graft Versus Host Disease
67
What is the only transplant that does not require immunosuppressive drugs for life?
successful bone marrow transplant
68
What factors can make Graft versus host disease milder?
Young pt
Good histocompatability match
Cord blood
Female
69
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?
Acute Graft Versus Host Disease
70
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?
Chronic Graft Versus Host Disease
71
Chronic Graft Versus Host disease will resemble what clinically?
Lichen planus or systemic sclerosis
72
Which manifests more orally, Chronic or Acute Graft versus host disease
Chronic (80%)
73
Beyond ulcers, what are 2 other oral manifestations of oral Graft Versus Host Disease?
Xerostomia due to immunologica destruction of salivary glands Mucocelles on soft palate (not as common)
74
Mucoceles, which are seen commonly on lips and buccal mucosa of young males, if seen on the soft palat, one should think what?
Graft vs host disease
75
If the ulcers last longer than 2 weeks beyond the 2 week post bone marrow transplant buffer zone have what?
Acute graft vs host disease
76
What is the Primary strategy for dealing with Graft versus host disease?
reduce or prevent occurrence with good histocompatability match
77
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
Psoriasis
78
What is THE classic and one of the most common example of an immunologically mediated condition?
Lupus Erythematosus
79
What is the most common of the collagen vascular and connective tissue disease in the US
Lupus Erythematosus
80
Do we need to know the histology of psoriasis?
Nope
81
Most immunologic diseases, including Lupus Erythematous, affect which gender predominantly?
Female (8 times more likely)
82
Systemic lupus is caused by increased activity of what part of the immune system?
humoral limb (B lymphocytes [stimulated by abnormally functioning T lymphocytes ?])
83
What are common findings of systemic lupus erythematosus?
```
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
What is the most significant aspect of systemic lupus erythematosus?
Kidney failure
85
What is another systemic mortality risk in systemic lupus erythematosus?
bacterial endocarditis
86
How can systemic lupus erythematous manifest orally?
Look lichenoid on palatal, buccal mucosa, and gingival. Ulcers, pain, erythema, hyperkeratosis
87
if Lupus manifests on the vermillion zone of the lower lip it is called what?
Lupus cheilitis
88
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?
Chronic cutaneous Lupus erythematosus
89
What is a lab test that is high in Lupus erythematous patients?
high in ANA = autonucleic antibody (higher than in any other disease)
90
What is the most common cause of death in systemic lupus erythematosus?
Renal failure
91
What are treatments for Lupus if mild?
Avoid sun
NSAIDs
Antimalarials (hydroxychloroquine)
92
What is the treatment for Lupus if severe?
corticosteroids and other immunosuppressives
93
If you see lichen planus, what else can be put on the differential?
Lupus erythematous
94
What is an mmunologically mediated deposition of dense collagen in tissues also known as Scleroderma?
Systemic sclerosis
95
What is the first sign of systemic sclerosis, but is not diagnostic for the disease?
Raynaud’s phenomenon
96
Systemic sclerosis higher in which gender?
Females
97
What is the cause of death in systemic sclerosis?
pulmonary fibrosis, leading to pulmonary hypertension and heart failure
98
What does pulmonary fibrosis do?
reduces lung surface area for oxygen exchange
99
What is the clinical appearance of a person with systemic sclerosis?
smooth, taut, mask-like face, nasal alae atrophy = mouse facies
100
What is the character of the mouth of pt with systemic sclerosis?
microstomia with purse-string furrows radiating from the mouth
101
What is the character of the gingiva in pt with systemic sclerosis?
Lose attached gingiva
102
What are symptoms of oral systemic sclerosis?
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
What is resorption of terminal phalnages and flexure contractures producing club like fingers in systemic sclerosis and some other diseases?
Acro-osteolysis
104
Does systemic sclerosis with its widened PDL and loss of attached gingival respond to periodontal therapy?
No
105
Siversky loves this disease and brought up the fact that this disease, radiographically, shows a loss of the lamina dura
Hyperparathyroidism
106
What is a systemic sclerosis that is localized usually leaving a scar called en coup de sabre, Dr, Siversky mentioned it as Morpha?
localized scleraderma
107
What can the pressure of collagen deposition cause orally and skeletally?
resorption of posterior ramus, coronoid process and condyle
108
What systemic medication is prescribed to try and inhibit collagen production in systemic sclerosis?
D-penicillamine
109
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?
slow the disease process
110
would it be smart to fabricate dentures for a systemic sclerosis patient?
No due to microstomia and inelasticity of the mouth
111
What does CREST stand for?
Calcinosis cutis Raynaud’s phenomenon Esophageal dysfunction Sclerodactyly Telangiectasias
112
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?
Calcinosis cutis
113
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?
Ranaud's phenomenon
114
Is Raynaud’s phenomenon indicative of scleraderma or CREST?
No
115
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.
Esophageal dysfunction
116
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?
Sclerodactyly
117
What part of CREST due to fragile vessels, and is similar to Hereditary Hemorrhagic Telangiectasia?
Telangiectasias
118
Which has better prognosis, CREST or systemic sclerosis/scleraderma?
CREST
