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)