Mucocutaneous Conditions Flashcards
1
Q
Inappropriate production of antibodies by the host directed against host tissue (autoantibodies)
A
Pemphigus vulgaris
2
Q
What do the autoantibodies destroy in Pemphigus vulgaris?
A
Desmosomes
3
Q
Role of desmosomes
A
Bond epithelial cells together
4
Q
Where can Pemphigus vulgaris be seen clinically?
A
Skin and oral lesions
5
Q
What is important about the oral lesions in Pemphigus vulgaris
A
“First to show, last to go” (first to show - hardest to treat)
6
Q
Describe the oral lesions in Pemphigus vulgaris
A
Superficial, ragged erosions and ulcerations
7
Q
Patients with Pemphigus vulgaris show a ____ ____ sign
A
Positive Nikolsky
8
Q
Nikolsky sign
A
Inducing a bulla by applying firm, lateral pressure to normally appearing skin
9
Q
What is really important in management of Pemphigus vulgaris
A
Plaque control
10
Q
What is characteristic of skin involvement of Pemphigus vulgaris
A
Flaccid cutaneous bulla
11
Q
Normal tissue adjacent to the ulceration or erosion in Pemphigus vulgaris should be sampled in a biopsy. What for? Where is the sample taken?
A
For direct immunofluoresent and light microscopic evaluation. Sample at the periphery, not the ulcerated center
12
Q
2 histological features of Pemphigus vulgaris
A
- Intraepithelial clefting above the basal layer (SUPRABASILAR)
- Acantholysis (breakdown of spinous layer)
13
Q
A technique that uses fluorescent-labeled antibodies to detect specific targets
A
Immunofluorescence
14
Q
What is DIRECT immunofluorescence used to detect?
A
Autoantibodies bound to patient’s tissues
15
Q
What is INDIRECT immunofluorescence used to detect?
A
Antibodies circulating in the blood
16
Q
Autoantibodies in Pemphigus vulgaris will bind desmosomal components ( desmoglein ___ & ___)
A
1 & 3
17
Q
Treatment for Pemphigus vulgaris
A
- Systemic corticosteroids
- OHI
- Prophy 3-4x per year
- SOFT toothbrush
18
Q
Why do topical corticosteroids show little effect in Pemphigus vulgaris?
A
Because Pemphigus vulgaris is a systemic disease
19
Q
Prognosis for Pemphigus vulgaris
A
Fatal if not treated (SEVERE infection, loss of fluids/electrolytes, malnutrition due to mouth pain)
20
Q
What may lead to mortality in Pemphigus vulgaris?
A
Complications of long-term steroids
21
Q
Condition that resembles Pemphigus vulgaris due to blister formation, but is 2x as common as Pemphigus vulgaris?
A
Mucous Membrane Pemphigoid (MMP)
22
Q
Another name for Mucous Membrane Pemphigoid
A
Cicatricial (scarring) Pemphigoid
23
Q
Are patients with Mucous Membrane Pemphigoid older or younger than patients with Pemphigus Vulgaris?
A
Older
24
Q
Does Mucous Membrane Pemphigoid have a male or female predilection?
A
Female
25
Where is scarring typically seen in patients with Mucous Membrane Pemphigoid
Skin, Symblephreron (conjunctiva), oral mucosa (rare)
26
Why might you see intact intraoral blisters in patients with Mucous Membrane Pemphigoid?
Because the split is subepithelial
27
Clinical feature seen in patients with Mucous Membrane Pemphigoid
Desquamative gingivitis
28
Desquamative gingivitis
A DESCRIPTIVE TERM - erythema, desquamation, ulceration
29
What is the most significant aspect of Mucous Membrane Pemphigoid
Ocular involvement of symblepheron
30
What does ocular scarring lead to in patients with Mucous Membrane Pemphigoid
Scarring obstructs the orifices of glands that produce tears, resulting in a dry eye
31
What does ocular dryness lead to in patients with Mucous Membrane Pemphigoid
Dryness leads to keratinization of the corneal epithelium, leading to blindness
32
Term for upper eyelid being turned inward
Entropion
33
Term for eyelashes rubbing against the eye
Trichiasis
34
What must you include in a biopsy for a patient with Mucous Membrane Pemphigoid
A generous sample of normal mucosa, 0.5-1.0 cm away from areas of ulceration/erythema
35
What type of cleft formation is seen in patients with Mucous Membrane Pemphigoid ?
SUBepithelial
36
Subepithelial cleft formation
Separation of the epithelium from the connective tissue at the basement membrane zone
37
What will you see in the immunopathology of patients with Mucous Membrane Pemphigoid
1. Linear deposition of immunoreactants at the basement membrane zone
2. Positive DIF, Negative IIF (few patients will have circulating autoantibodies)
38
Treatment for patient with Mucous Membrane Pemphigoid when only oral lesions are present
Topical steroids, tetracycline/niacinamide or dapsone, frequent prophy visits (every 3-4 months)
39
Treatment for patient with Mucous Membrane Pemphigoid if there is ocular involvement
Systemic immunosuppressive therapy
40
Prognosis for patients with Mucous Membrane Pemphigoid
Blindness from untreated ocular disease, condition can usually be controlled
41
Most common of the autoimmune blistering conditions
Bullous Pemphigoid
42
Where is Bullous Pemphigoid primarily seen?
Skin
43
Early symptom of Bullous Pemphigoid? Followed by?
Pruritus followed by multiple, tense bullae, blisters on normal or erythematous skin
44
Describe the transformation of Bullous Pemphigoid lesions?
Rupture, crust, heal without scarring
45
Oral involvement in Bullous Pemphigoid is _____ (common/uncommon), and the bullae rupture ____ (later/sooner) than on the skin
Uncommon
| Sooner
46
What is left behind when oral bullae rupture in patients with Bullous Pemphigoid
Large, shallow ulcerations with smooth, distinct margins
47
Is Bullous Pemphigoid seen supraepithelial or subepithelial? What is that similar to?
SUBepithelial - similar to Mucous Membrane Pemphigoid
48
What do you see in the immunopathology of patients with Bullous Pemphigoid
Positive DIF AND IIF with immunoreactants deposited at the basement membrane
49
Treatment for Bullous Pemphigoid
Management is similar to cicatricial pemphigoid, but most resolve spontaneously in 1-2 years
50
When might problems arise in patients with Bullous Pemphigoid
With use of immunosuppressive therapy in older patients
51
Acute onset ulcerative disorder of the skin and mucous membranes in younger patients, most have an unknown etiology
Erythema Multiforme
52
Although most cases of Erythema Multiforme have an unknown etiology, what are some known etiologies ?
Preceding infection (viral (herpes), bacterial (mycoplasma pneumoniae)), and Medication related (antibiotics and analgesics)
53
When do patients with Erythema Multiforme experience prodromal symptoms? What are they?
1 week before onset (fever, malaise, headache, cough, sore throat)
54
2 degrees of severity in patients with Erythema Multiforme
1. EM minor
| 2. EM major
55
Where is EM minor seen?
Skin (extremities) and Mucosa (oral, conjunctival, genitourinary, respiratory)
56
What will you see on the vermilion zones of patients with EM minor?
Hemorrhagic crusting
57
2 skin features that you may see in patients with EM minor?
1. Round, dusky-red patches on skin of extremities "targeted lesions"
2. Bullae with necrotic centers
58
Oral features seen in patients with EM minor? Which areas are usually spared?
Erythematous patches of oral mucosa that undergo necrosis and result in large, shallow erosions and ulcers with irregular borders (gingiva and hard palate usually spared)
59
Clinical features of EM major
2 or more mucosal sites in conjunction with skin lesions, and ocular involvement that can produce symblepheron
60
Stevens'-Johnson syndrome
At least 2 mucosal sites plus skin involvement
61
Toxic epidermal necrolysis (Lyell's disease)
Difuse bullous involvement of skin and mucosa
62
Difference in Stevens' Johnson syndrome and Toxic Epidermal Necrolysis?
Degree of skin involvement and patient age
SJS (< 10% and younger patients)
TEN (>30% and older patients)
63
Difference in SJS/TEN and Erythema Multiforme
1. SJS/TEN almost always triggered by a drug (rather than infection)
2. SJS/TEN skin lesions begin as red macules on trunk (rather than extremities)
64
3 clinical features of SJS/TEN
1. Skin sloughing and flaccid bullae develop within 2 weeks
2. Patients appear badly scalded - treated in burn unit
3. Almost all patients have mucosal involvement (esp. oral)
65
How to diagnose EM, SJS, TEN
Usually based on clinical presentation (DIF and IIF non-specific)
66
Where are the vesicles seen in the histology of EM, SJS, and TEN?
Subepithelial or intraepithelial
67
Treatment for Erythema Multiforme
Discontinue causative drug, IV re-hydration, topical anesthetic/analgesic (steroid benefits are controversial)
68
What medication has been implicated in inducing TEN?
NSAIDS
69
What medication is avoided in the management of TEN? WHY?
Steroids - associated with increased mortality
70
What treatment is seen to be helpful in patients with TEN?
IV administration of pooled human immunoglobulins (blocking apoptosis of epithelial cells)
71
Prognosis for EM minor and major
Self-limiting in 2-6 weeks, but 20% get recurrences
72
Which has a higher mortality rate, SJS or TEN?
TEN
73
If a patient survives SJS or TEN, the ___ heals in 3-5 weeks, _____ lesions take longer, and ____ damage is common
Skin
Oral
Ocular
74
2 additional names for Erythema Migrans
1. Geographic tongue
| 2. Benign migratory glossitis
75
One of the more common oral conditions, occurring in 1-3% of the population - considered a "common oral lesion"
Erythema Migrans
76
What is the erythema in Erythema Migrans due to?
Atrophy of filiform papillae and shearing off of the parakeratin - remaining epithelium thins and appears red
77
Erythema migrans occurs in 1/3 of patients who already have ____ ____
Fissured tongue
78
Clinical feature of Erythema Migrans
Wax/Wane and Heal/Develop in different areas
79
Where is Erythema Migrans typically seen
Dorsal and lateral anterior 2/3 of the tongue
80
What is Erythema Migrans called when it is seen on other non-keratinized mucosal surfaces?
Ectopic Geographic Tongue
81
Multiple, well-demarcated zones of erythema surrounded at least partially by a slightly elevated yellow-white serpentine or scalloped border
Erythema migrans
82
Term when Erythema migrans is singular
Persistens
83
Histologically, Erythema Migrans is similar to ____
Psoriasis
84
Description of Erythema Migrans on a biopsy report
"Psoriasiform mucositis"
85
Erythema migrans shows extensive ______ formation in the superficial spinous layer
Microabscess (neutrophils)
86
What causes an erythematous macule in Erythema Migrans?
Neutrophilic microabsesses lift the keratin which then falls off and leaves the erythematous macule
87
Treatment for Erythema migrans
Generally none - maybe a topical steroid if there is sensitivity
88
Purple, polygonal, pruritic papules with Wickham's striae (lacy white lines)
Cutaneous Lichen Planus
89
Mucosal Lichen Planus is typically managed as a ___ ___
Chronic condition
90
Lichen Planus (Oral and Cutaneous) has a ____ predilection
Female
91
Condition seen on the flexor surfaces of wrists, lumbar region, shins
Cutaneous Lichen Planus
92
2 forms of Oral Lichen Planus
1. Reticular (lacy white lines)
| 2. Erosive (ELP) - erythematous, may ulcerate
93
True/False: Oral Lichen Planus always occurs with skin lesions
False - it can occur alone or with skin lesions
94
What is the most common form of oral lichen planus?
Reticular
95
What is the most symptomatic form of oral lichen planus?
Erosive (esp. with acidic, salty, or spicy foods)
96
Where is oral lichen planus typically seen?
Bilateral buccal mucosa, tongue, gingiva (but any intraoral surface and lips)
97
If the dorsal tongue is involved in oral lichen planus, what might it appear as?
Patchy keratosis and atrophy
98
What may be superimposed on oral lichen planus?
Candida albicans
99
If candida albicans is superimposed, what will happen to oral ulcerative conditions?
The conditions worsen
100
What is significant about the rete ridges associated with oral lichen planus?
They are absent or pointed ("saw toothed")
101
For Oral lichen planus, DIF is usually non-specific. What is the exception?
To rule out other immune-mediated conditions
102
What can have the same hisology response as lichen planus?
Inflammatory response to epithelial dysplasia (especially mild).
103
What kind of diagnosis is oral lichen planus?
Clinical
104
What should be ruled out in oral lichen planus?
Candidiasis
105
Treatment for reticular lichen planus?
None
106
Treatment for erosive lichen planus?
Potent topical steroid (NOT systemic)
107
Why would you biopsy an oral lichen planus lesion?
If it is suspected to be pre-malignant or malignant
108
Prognosis for oral lichen planus
Varies - some well controlled and some difficult to control
109
Topical steroids predispose a patient to _____
Candida
110
What is a recurring challenge in patients with oral lichen planus?
Candidiasis (due to dry mouth, dentures, ATB, inhaler use)
111
A number of conditions that mimic oral lichen planus (drug reaction, amalgam reaction, mucosal cinnamon reaction, etc.)
Lichenoid mucositis
112
Most common collagen vascular/connective tissue disease in the US
Lupus erythematosus
113
3 forms of Lupus erythematosus
1. Chronic cutaneous lupus erythematosus (CCLE)
2. Systemic lupus erythematosus (SLE)
3. Subacute cutaneous lupus erythematosus (intermediate between CCLE and SLE)
114
What is the appearance of skin lesions for patients with Chronic cutaneous lupus erythematosus
Scaly, erythematous patches in sun-exposed areas
115
Describe the healing of Chronic cutaneous lupus erythematosus lesions
Heal and then reappear in a different area
| May result in atrophy and scarring with hypo/hyperpigmentation
116
Mucosal lesions of Chronic cutaneous lupus erythematosus are essentially identical to ___ ____ __, but seldom occurs without _____ lesions
Erosive lichen planus
| Skin
117
Form of lupus erythematosus that will wax/wane and has a female predilection
Systemic
118
Form of lupus erythematosus that affects women of color more than white women?
Systemic
119
What is the initial manifestation of systemic lupus erythematosus
Protean manifestation (fever, weight loss, arthritis, fatigue, general malaise)
120
What area of the face is not affected by the butterfly rash associated with systemic lupus erythematosus
The nasolabial fold
121
What causes skin lesions to flare in patients with systemic lupus erythematosus
UV exposure
122
What is the most significant aspect of the disease in many patients with systemic lupus erythematosus
Renal involvement
123
What is another common involvement in patients with systemic lupus erythematosus
Cardiac involvement
124
Systemic lupus erythematosus found on the vermilion zones is called ___ ____
Lupus cheilitis
125
What may help lead to the diagnosis in CCLE and SLE
Subepithelial edema
126
What is characteristic and helps in the diagnosis of CCLE?
Skin lesions
127
How do you diagnose SLE
Criteria by American Rheumatism Association for clinical and lab findings
128
Serum studies show anti-nuclear antibodies in most patients with ____ but it is negative in patients with ____
SLE
| CCLE
129
Measures the amount and pattern of antibodies in your blood that work against your own body; a non-specific finding (but a screening tool)
ANA
130
Deposition of immunoreactants at the basement membrane zone of normal skin
Positive lupus band test
131
Positive lupus band tests are non-specific in patients with _____
SLE
132
Sunscreen, NSAIDS, and antimalarial drug therapy can all help in the treatment of _____
Lupus erythematosus
133
Systemic steroids, immunomodulating agents, and even kidney transplants may be needed for treatment of _____
SLE
134
Prognosis for CCLE
Good - most resolve, some will transform to CCLE and SLE
135
Prognosis for SLE
Worse for men than women
Worse for blacks than whites
Depends on organs affected
136
Most common cause of death in patients with SLE
Renal failure
137
Relatively rare condition characterized by inappropriate deposition of dense collagen (replaces/destroys normal tissue)
Systemic sclerosis
138
Another term for systemic sclerosis
Scleroderma ("hard" "skin")
139
Diffuse, hard, smooth texture of skin is seen in patients with ____ ____
Systemic sclerosis
140
Raynaud phenomenon, Sclerodactyly, and Acro-osteolysis may be seen in patients with ___ ____
Systemic sclerosis
141
Patients with this condition are known for having a "mask-like" face, atrophy of alae, and mouse facies (pinched appearance of nose)
Systemic sclerosis
142
Systemic sclerosis has a ____ predilection
Female
143
Discoloration of the fingers/toes after exposure to changes in temperature or emotional events causing spasm of blood vessels
Raynaud's phenomenon
144
Is Raynaud's phenomenon specific to systemic sclerosis?
No
145
What causes the claw-like deformity (sclerodactyly) of fingers in patients with systemic sclerosis?
The collagen deposition
146
Destruction of the digit tips, including bone
Acro-osteolysis
147
Why might patients with systemic sclerosis have dysphagia?
Due to esophageal involvement
148
Limitation in opening results in ____ in patients with systemic sclerosis
Microstomia (Purse-string appearance)
149
Radiographically - widening of PDL, resorption of the posterior ramus/condyle/coronoid/chin, possible root resorption
Systemic sclerosis
150
The autoantibodies in systemic sclerosis are directed against _____
Scl-70
151
What kind of antibodies are seen in limited cutaneous systemic sclerosis?
Anticentromere
152
ACE inhibitors for HTN, esophageal dilation, OHI are used as treatment for ____ ____
Systemic sclerosis
153
Prognosis for Systemic sclerosis
Death due to internal organ deposition of collagen (worse for cardiac involvement, most death due to pulmonary involvement)
154
CREST Syndrome - what does CREST stand for?
```
Calcinosis cutis
Raynaud's phenomenon
Esophageal dysfunction
Sclerodactyly
Telangiectasia
```
155
Milder variant of systemic sclerosis
CREST syndrome
156
CREST syndrome has a ____ predilection
Female
157
Subcutaneous nodules that are a deposition of calcium salts
Calcinosis cutis
158
Treatment for CREST syndrome is similar to ___ ___
Systemic sclerosis
159
Which has a better prognosis: CREST syndrome or systemic sclerosis?
CREST syndrome
