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Dermatoses And Pigmentary Disorders Flashcards

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1
Q

If a pt is diagnosed as having oral mucous membrane pemphigoid, what referral must also be made

A

All pts diagnosed with oral mucous membrane pemphigoid also need a consultant opthalmic opinion

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2
Q

When must a biopsy of a localised pigmented lesion be taken? All the time?

A

No only when the pigmented lesion is enlarging or darkening in appearance

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3
Q

What is the relationship between skin and oral mucosal lesions?

A

There’s a developmental similarity between the skin and oral mucosa
So pathological conditions may occur simultaneously.
A number of mucocutaneuos disorders may initially present as oral signs and symptoms

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4
Q

Can lichen planus and Lichenoid reactions have cutaneous involvement?

A

Yes
Lichen planus skin lesions are “dusky pink Papules” commonly found on wrists forearms and legs
Lichenoid reactions are variable, maybe erythematous areas of the skin at any site

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5
Q

What are the cutaneous lichen planus lesions presentations?

A

Lichen planus skin lesions are “dusky pink Papules” commonly found on wrists forearms and legs

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6
Q

What are fixed drug eruptions?

A

This is where systemic administrarion of a drug produces cutaneous changes at the same site on each occasion it is given.

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7
Q

What is the term given when systemic administrarion of a drug produces cutaneous changes at the same site on each occasion it is given.

A

Fixed drug eruption

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8
Q

How does pemphigus present? What are the 4 forms of pemphigus

A 
There are 4 forms of pemphigus
Pemphigus vulgaris
Pemphigus foliaceous
Pemphigus vegetans
Pemphigus erythematosus
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9
Q

What is the most common pemphigus

A

P vulgaris is the most common

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10
Q

What is the epidemiology of pemphigus

A

Pemphigus is rare

Affects middle aged or elderly

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11
Q

What is the clinical onset of pemphigus

A

Insidious, developing over several weeks and approx half of 5he cases initially present as oral lesions

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12
Q

What are the early features of pemphigus

A

Non specific mucosal erosion

Soon accompanied by cutaneous changes

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13
Q

How is the clinical diagnosis of pemphigus confirmed

A

Histopathological exam
Routine formalin fixed tissue
And direct and indirect immunofluorescence investigation of a fresh frozen sample
Blood sample

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14
Q

What solution is used to fix a tissue biopsy

A

10% formalin

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15
Q

What will the histopathological exam of pemphigus reveal

A

Intra epithelial split on routine tissue and direct immunofluorescence
Revealing and inter cellular deposition of igG in the epithelium “fish net” appearance

Blood sample will reveal amount of circulating antibody using indirect immunofluorescence

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16
Q

What is meant by “fish net” appearance of the epithelium?

A

In pemphigus, there is an intra epithelial split, inter cellular deposition of igG in the epithelium

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17
Q

What is pemphigus

A

Vesiculo bullous disorder
Rare but potentially fatal
May initially present as oral lesions

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18
Q

Is possible to predict which individuals with oral pemphigus will subsequently develop extensive skin involvement?

A

No

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19
Q

Why does the pt require immediate hospitalisation if they are diagnosed with pemphigus

A

The serious consequences of rapid alterations of protein and electrolyte balance

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20
Q

How is pemphigus treated

A

High dose presnisolone 50 - 60mg once daily

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21
Q

Apart from the systemic corticosteroid therapy with presnisolone what else can be considered to treat pemphigus

A

Immunomodulatory drug azathioprine 50 - 10mg daily since this has the advantage of allowing the amount of steroid therapy to be reduced

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22
Q

What is used to monitor the effectiveness of the tx? Why?

A

Indirect immunofluorescence

The serum titre of intracellular adhesion auto antibody desmoglein 3 reflects mucosal disease activity

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23
Q

Can pemphigus be induced by drugs?

A

Yes
Various drugs can induce pemphigus and that pemphigus can occur in pts with malignant disease so called paraneoplastic pemphigus

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24
Q

What is pemphigoid

A

Deep vesiculo bollous disorder

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25
What are the different forms of pemphigoid
There are two forms of pemphigoid Bollous pemphigoid Mucous membrane pemphigoid
26
What is bullous pemphigoid how does it present?
These lesions dominate the clinical involvement whilst in mucous membrane pemphigoid mucosal tissues are usually affected and cutaneous involvement occurs infrequently
27
What are the oral lesions of mucous membrane pemphigoid caused by
Sub epithelial blistering | Most often seen as irregular areas of ulceration or desquamataive gingibitis
28
Occasionally a blood blistering may be seen in mucous membrane pemphigoid. True or false?
True Occasionally a blood blistering may be seen in mucous membrane pemphigoid
29
What does histopathological exam of a pt with pemphigoid show
Sub epithelial split on routine tissue and direct immunofluorescence will show a linear deposition of igG and complement in the basal region
30
What will biopsy of pemphigoid show
linear deposition of: 1. igG 2. complement in the basal region
31
What is the tx for mucous membrane pemphigoid
Topical corticosteroids Eg | Betamethosone clobetasol, fluticasone or fluocinolone
32
What are the topical corticosteroids used to tx pemphigoid
Betamethosone clobetasol, fluticasone or fluocinolone
33
What are Betamethosone clobetasol, fluticasone and fluocinolone all used to tx
Pemphigoid
34
What is used in a vacuum formed appliance for 5 minutes in the morning and 5 minutes in the evening on gingival pemphigoid lesions
Fluocinolone acetonide cream
35
If there is extensive gingival involvement what may be used as a steroid adjunct to treat pemphigoid
Azathioprine Ciclosporin (immunosuppressant medications) Dapsone (an antibiotic)
36
What two immunosuppressant medications can be used to treat extensive pemphigoid
Azathioprine | Ciclosporin
37
What is Dapsone
This is an antibiotic that may be used to treat extensive pemphigoid
38
What combination of drugs may be used to treat severe pemphigoid
Steroids Immunosuppressants Antibiotics
39
What is fluocinolone acetonide
Fluocinolone acetonide is a corticosteroid primarily used in dermatology to reduce skin inflammation and relieve itching. It is a synthetic hydrocortisone derivative
40
What is prednisolone
Prednisolone is a steroid medication used to treat certain types of allergies, inflammatory conditions, autoimmune disorders, and cancers. Some of these conditions include adrenocortical insufficiency, high blood calcium, rheumatoid arthritis, dermatitis, eye inflammation, asthma, and multiple sclerosis. 
41
What is prednisone
Prednisone is a glucocorticoid medication mostly used to suppress the immune system and decrease inflammation in conditions such as asthma, COPD, and rheumatologic diseases. It is also used to treat high blood calcium due to cancer and adrenal insufficiency along with other steroids. It is taken by mouth. 
42
What is the difference between prednisone and prednisolone
The main difference between prednisone and prednisolone is that prednisone must be converted by liver enzymes to prednisolone before it can work. In people with severe liver disease, prednisolone is usually preferred
43
What is Dapsone usually used with
Dapsone is an antibiotic commonly used in combination with rifampicin and clofazimine
44
What should the clinician warn the pt of when prescribing Dapsone? What may be a side effect
The most prominent side-effects of this drug are dose-related hemolysis (which may lead to hemolytic anaemia) and methaemaglobinaemia
45
What accompanies the oral lesions of pemphigoid? What is the textbook systemic manifestation?
Ocular disease | Symblepharon and glaucoma
46
What is sympblepharon
partial or complete adhesion of the palpebral conjunctiva of the eyelid to the bulbar conjunctiva of the eyeball.
47
What is glaucoma
Glaucoma is a group of eye diseases which result in damage to the optic nerve and cause vision loss Vision loss from glaucoma, once it has occurred, is permanent
48
What is the usual clinical history of angina bullosa haemorrhagica
Solitary blister which develops within seconds usually on the soft palate during eating although in some cases the blister arises spontaneously The blister will usually burst spontaneously within 24hr leaving an ulcerated area surrounded by evidence of haemorrhage
49
What are the synonyms for angina bullosa haemorrhagica
Abh (recurrent intra oral blood blisters)
50
Why is angina bullosa haemorrhagica named 'angina' isnt this misleading
The lesions can be sufficiently large to cause a sensation of choking hence the use of the term angina
51
How does the ulcerated area(s) seen in ABH form
The blister will usually burst spontaneously within 24hr leaving an ulcerated area surrounded by evidence of haemorrhage
52
What is the pathological bases for ABH
The pathological basis for ABH is not known | No immunological abnormality has been found
53
Is ABH linked with any immunological abnormality
No.
54
What may a predisposing factor for ABH (not proven)
A history of steroid therapy either systemic or inhaler
55
Is ABH related in any way to mucous membrane pemphigoid
Histopathological exam of an intact blood filled blister reveals features identical to MMP but immunofluorescence is negative for ABH
56
What is the DDx for ABH? What condition Must be excluded
Thrombocytopenia
57
What should be done if a pt presents to the oral med department with a blood filled bulla
If the history is not classical of ABH them a full blood count to exclude thrombocytopenia is mandatory for patients presenting with a blood filled bulla
58
What is the tx for ABH
Reasurrance | No active treatment
59
Does ABH resolve
Usually there is total resolution of the lesion within 7-10 days
60
Does ABH remit?
Yes usually
61
What is the pattern of occurrence of ABH
Generally pts suffer 5-10 episodes over 2-3 years and the condition then remits spontaneously
62
In angina bullosa haemorrhagica Generally pts suffer ____-___episodes over ___-___ years and the condition then remits spontaneously
5,10 | 2,3
63
What is lupus
Lupus is a long-term autoimmune disease in which the body's immune system becomes hyperactive and attacks normal, healthy tissue. Symptoms include inflammation, swelling, and damage to the joints, skin, kidneys, blood, heart, and lungs
64
What are the different forms of lupus
Systemic or discoid
65
What is SLE
Systemic lupus erythematousus Occurs in women under 30 years old Can effect most systems skin kidney brain and salivary tissues
66
Who does SLE effect
Women under 30 years old
67
When the salivary glands are affected SLE forms the connective tissue component of what syndrome?
SJOGRENS
68
Almost of patients with SLE have high titres of what factor?
Circulating anti nuclear factor
69
What is DLE
Discoid lupus erythematousus Women>men 40s and 50s age group
70
Who does DLE effect
Women>men | 40s and 50s age group
71
Where are DLE lesions found
Anywhere on the skin although most commonly the ear
72
What system is affected by DLE
Skin
73
Who is most vulnerable to DLE (social history)
Those exposed to most sunlight
74
How does lupus affect dental treatment
Care and attention required due to pt tissue biotype likely being sensitive Symptomatic relief Oral lesions consisting of white patches which resemble lichen planus may occur and require symptom relief - topical steroid preparations
75
What may be the oral manifestations of lupus
Sjogrens syndrome--dry mouth xerostomia White patches resembling lichen planus
76
What is psoriasis
Psoriasis is a skin condition that causes red, flaky, crusty patches of skin covered with silvery scales. These patches normally appear on your elbows, knees, scalp and lower back, but can appear anywhere on your body.  Most people are only affected with small patches. In some cases, the patches can be itchy or sore. Psoriasis affects around 2% of people in the UK. It can start at any age, but most often develops in adults under 35 years old, and affects men and women equally. The severity of psoriasis varies greatly from person to person. For some it's just a minor irritation, but for others it can majorly affect their quality of life. Psoriasis is a long-lasting (chronic) disease that usually involves periods when you have no symptoms or mild symptoms, followed by periods when symptoms are more severe. Why it happens People with psoriasis have an increased production of skin cells. Skin cells are normally made and replaced every 3 to 4 weeks, but in psoriasis this process only takes about 3 to 7 days. The resulting build-up of skin cells is what creates the patches associated with psoriasis. Although the process is not fully understood, it's thought to be related to a problem with the immune system. The immune system is your body's defence against disease and infection, but it attacks healthy skin cells by mistake in people with psoriasis. Psoriasis can run in families, although the exact role genetics plays in causing psoriasis is unclear. Many people's psoriasis symptoms start or become worse because of a certain event, known as a trigger. Possible triggers of psoriasis include an injury to your skin, throat infections and using certain medicines. The condition is not contagious, so it cannot be spread from person to person. Diagnosed by biopsy
77
What is epidermolysis bullosa
Epidermolysis bullosa (EB) is the name for a group of rare inherited skin disorders that cause the skin to become very fragile. Any trauma or friction to the skin can cause painful blisters. Symptoms of epidermolysis bullosa The main symptoms of all types of EB include: skin that blisters easily blisters inside the mouth blisters on the hands and soles of the feet scarred skin, sometimes with small white spots called milia thickened skin and nails Types of epidermolysis bullosa The 3 main types of EB are: epidermolysis bullosa simplex (EBS) – the most common type, which tends to be milder with a low risk of serious complications dystrophic epidermolysis bullosa (DEB) – which can range from mild to severe junctional epidermolysis bullosa (JEB) – the rarest and most severe type The type reflects where on the body the blistering takes place and which layer of skin is affected. There are also many variants of these 3 main types of EB, each with slightly different symptoms.
78
What is EBA
aquisita form of EB Affects the digestive tract Only appears in late life Managed by symptom relief
79
What is linear Iga disease? Why is it called this
Linear IgA bullous disease is an autoimmune blistering disease in which blisters form in the skin and mucous membranes. Its name comes from the characteristic findings on direct immunofluorescence of a skin biopsy, in which a line of IgA antibodies can be found just below the epidermis.
80
What is dermatitis herpetiformis
Although not a symptom of coeliac disease, if you have an autoimmune response to gluten, you may develop a type of skin rash called dermatitis herpetiformis. The rash is itchy and has blisters that burst when scratched. It usually occurs on your elbows, knees and buttocks, although it can appear anywhere on your body. It's estimated that around 1 in 5 people with coeliac disease also develop dermatitis herpetiformis. The exact cause of dermatitis herpetiformis is not known, but, as with coeliac disease, it's associated with gluten. Like coeliac disease, it should clear up after switching to a gluten-free diet.
81
Define Dermatosis
Any disease of the skin Eg pemphigus pemphigoid, ABH, lupus, linear igA disease, dermatitis herpetiformis, epidermolysis bullosa
82
What other condition (in the mouth) has psoriasis been compared to histologically
Geographic tongue
83
What is the link between psoriasis and geographic tongue
It has been claimed that geographic tongue occurs more frequently in the guttate form of psoriasis
84
What is the link between psoriasis and geographic tongue
It has been claimed that geographic tongue occurs more frequently in the guttate form of psoriasis
85
Define guttate
resembling drops or characterized by markings that resemble drops.
86
What is guttate psoriasis
Guttate psoriasis appears as small, round spots called papules [PAP-yules] that are raised and sometimes scaly. Guttate lesions usually appear on the arms, legs and torso, with rare cases forming in the scalp, face and ears. Guttate psoriasis often develops suddenly and may appear after an infection like strep throat. It is a good idea to consult with your health care specialist to be checked for strep throat if you have guttate psoriasis, as that infection can occur without any obvious symptoms.
87
Is geographic tongue symptomatic
Geographic tongue may either be asymptomatic or produce a complaint of discomfort on eating hot or spicy foods
88
What may exacerbate geographic tongue
Eating hot or spicy foods Certain acids Lack of vitamins in the diet

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