Dermatoses And Pigmentary Disorders Flashcards
If a pt is diagnosed as having oral mucous membrane pemphigoid, what referral must also be made
All pts diagnosed with oral mucous membrane pemphigoid also need a consultant opthalmic opinion
When must a biopsy of a localised pigmented lesion be taken? All the time?
No only when the pigmented lesion is enlarging or darkening in appearance
What is the relationship between skin and oral mucosal lesions?
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
Can lichen planus and Lichenoid reactions have cutaneous involvement?
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
What are the cutaneous lichen planus lesions presentations?
Lichen planus skin lesions are “dusky pink Papules” commonly found on wrists forearms and legs
What are fixed drug eruptions?
This is where systemic administrarion of a drug produces cutaneous changes at the same site on each occasion it is given.
What is the term given when systemic administrarion of a drug produces cutaneous changes at the same site on each occasion it is given.
Fixed drug eruption
How does pemphigus present? What are the 4 forms of pemphigus
There are 4 forms of pemphigus Pemphigus vulgaris Pemphigus foliaceous Pemphigus vegetans Pemphigus erythematosus
What is the most common pemphigus
P vulgaris is the most common
What is the epidemiology of pemphigus
Pemphigus is rare
Affects middle aged or elderly
What is the clinical onset of pemphigus
Insidious, developing over several weeks and approx half of 5he cases initially present as oral lesions
What are the early features of pemphigus
Non specific mucosal erosion
Soon accompanied by cutaneous changes
How is the clinical diagnosis of pemphigus confirmed
Histopathological exam
Routine formalin fixed tissue
And direct and indirect immunofluorescence investigation of a fresh frozen sample
Blood sample
What solution is used to fix a tissue biopsy
10% formalin
What will the histopathological exam of pemphigus reveal
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
What is meant by “fish net” appearance of the epithelium?
In pemphigus, there is an intra epithelial split, inter cellular deposition of igG in the epithelium
What is pemphigus
Vesiculo bullous disorder
Rare but potentially fatal
May initially present as oral lesions
Is possible to predict which individuals with oral pemphigus will subsequently develop extensive skin involvement?
No
Why does the pt require immediate hospitalisation if they are diagnosed with pemphigus
The serious consequences of rapid alterations of protein and electrolyte balance
How is pemphigus treated
High dose presnisolone 50 - 60mg once daily
Apart from the systemic corticosteroid therapy with presnisolone what else can be considered to treat pemphigus
Immunomodulatory drug azathioprine 50 - 10mg daily since this has the advantage of allowing the amount of steroid therapy to be reduced
What is used to monitor the effectiveness of the tx? Why?
Indirect immunofluorescence
The serum titre of intracellular adhesion auto antibody desmoglein 3 reflects mucosal disease activity
Can pemphigus be induced by drugs?
Yes
Various drugs can induce pemphigus and that pemphigus can occur in pts with malignant disease so called paraneoplastic pemphigus
What is pemphigoid
Deep vesiculo bollous disorder
What are the different forms of pemphigoid
There are two forms of pemphigoid
Bollous pemphigoid
Mucous membrane pemphigoid
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
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
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
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
What will biopsy of pemphigoid show
linear deposition of:
1. igG
2. complement
in the basal region
What is the tx for mucous membrane pemphigoid
Topical corticosteroids Eg
Betamethosone clobetasol, fluticasone or fluocinolone
What are the topical corticosteroids used to tx pemphigoid
Betamethosone clobetasol, fluticasone or fluocinolone
What are Betamethosone clobetasol, fluticasone and fluocinolone all used to tx
Pemphigoid
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
If there is extensive gingival involvement what may be used as a steroid adjunct to treat pemphigoid
Azathioprine
Ciclosporin
(immunosuppressant medications)
Dapsone (an antibiotic)
What two immunosuppressant medications can be used to treat extensive pemphigoid
Azathioprine
Ciclosporin
What is Dapsone
This is an antibiotic that may be used to treat extensive pemphigoid
What combination of drugs may be used to treat severe pemphigoid
Steroids
Immunosuppressants
Antibiotics
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
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.
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.
What is the difference between prednisone and prednisolone
The main difference betweenprednisone and prednisoloneis thatprednisonemust be converted by liver enzymes toprednisolonebefore it can work. In people with severe liver disease,prednisoloneis usually preferred
What is Dapsone usually used with
Dapsone is an antibiotic commonly used in combination with rifampicin and clofazimine
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-relatedhemolysis(which may lead to hemolytic anaemia) andmethaemaglobinaemia
What accompanies the oral lesions of pemphigoid? What is the textbook systemic manifestation?
Ocular disease
Symblepharon and glaucoma
What is sympblepharon
partial or complete adhesion of the palpebral conjunctiva of the eyelid to the bulbar conjunctiva of the eyeball.
What is glaucoma
Glaucomais a group of eye diseases which result in damage to theoptic nerveand causevision loss
Vision loss from glaucoma, once it has occurred, is permanent
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
What are the synonyms for angina bullosa haemorrhagica
Abh (recurrent intra oral blood blisters)
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
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
What is the pathological bases for ABH
The pathological basis for ABH is not known
No immunological abnormality has been found
Is ABH linked with any immunological abnormality
No.
What may a predisposing factor for ABH (not proven)
A history of steroid therapy either systemic or inhaler
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
What is the DDx for ABH? What condition Must be excluded
Thrombocytopenia
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
What is the tx for ABH
Reasurrance
No active treatment
Does ABH resolve
Usually there is total resolution of the lesion within 7-10 days
Does ABH remit?
Yes usually
What is the pattern of occurrence of ABH
Generally pts suffer 5-10 episodes over 2-3 years and the condition then remits spontaneously
In angina bullosa haemorrhagica
Generally pts suffer ____-___episodes over ___-___ years and the condition then remits spontaneously
5,10
2,3
What is lupus
Lupusis 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
What are the different forms of lupus
Systemic or discoid
What is SLE
Systemic lupus erythematousus
Occurs in women under 30 years old
Can effect most systems skin kidney brain and salivary tissues
Who does SLE effect
Women under 30 years old
When the salivary glands are affected SLE forms the connective tissue component of what syndrome?
SJOGRENS
Almost of patients with SLE have high titres of what factor?
Circulating anti nuclear factor
What is DLE
Discoid lupus erythematousus
Women>men
40s and 50s age group
Who does DLE effect
Women>men
40s and 50s age group
Where are DLE lesions found
Anywhere on the skin although most commonly the ear
What system is affected by DLE
Skin
Who is most vulnerable to DLE (social history)
Those exposed to most sunlight
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
What may be the oral manifestations of lupus
Sjogrens syndrome–dry mouth xerostomia
White patches resembling lichen planus
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 ormild symptoms, followed by periods when symptoms are more severe.
Why it happens
People with psoriasis have anincreased production of skin cells.
Skin cells are normallymade 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 thoughtto be related to a problem with the immune system.
The immune systemis 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 includean 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
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.
What is EBA
aquisita form of EB
Affects the digestive tract
Only appears in late life
Managed by symptom relief
What is linear Iga disease? Why is it called this
Linear IgAbullousdiseaseis an autoimmune blisteringdiseasein 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 ofIgAantibodies can be found just below the epidermis.
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.
Define Dermatosis
Any disease of the skin Eg pemphigus pemphigoid, ABH, lupus, linear igA disease, dermatitis herpetiformis, epidermolysis bullosa
What other condition (in the mouth) has psoriasis been compared to histologically
Geographic tongue
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
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
Define guttate
resembling drops or characterized by markings that resemble drops.
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.
Is geographic tongue symptomatic
Geographic tongue may either be asymptomatic or produce a complaint of discomfort on eating hot or spicy foods
What may exacerbate geographic tongue
Eating hot or spicy foods
Certain acids
Lack of vitamins in the diet
