Bullous Disorders Flashcards
What are bullous disorders
Autoimmune diseases where damage to adhesion mechanisms in the skin results in blistering at various levels
What is bullous pemphigoid
Split is Deeper through the DEJ
What is pemphigus
Split is more superfical, intra - epidermal
Is dermatitis herpetiformis a type of a bullous disorder
Yes
What is dermatitis herpetiformis
Associated with gluten sensitive enteropathy
Itchy, vesicles removed by scratching leaving erosions
Symmetrical on scalp, shoulders, elbows, knees, ass
What is the nikolsky’s sign
Differentiates between pemphigoid and pemphigus
The tops layers of the skin slips away from the lower layers when slightly rubbed = indicates WITHIN THE EPIDERMIS IE PEMPHIGUS
Who is more commonly gets bullous pemphigoid
Elderly
Where do you get bullous pemphigoid
Localised to one areas or widespread on trunk and proximal limb
Features of bullous pemphigoid
Large tense Bullare
Blisters burst to leave erosions
Non - scarring
Nikolsky sign is negative
Where do you get pemphigus vulgaris
Scalp, face, axialle, groin
Features of pemphigus vulgaris
Flaccid vesicles, Bullae - thin roofed
Lesions rupture to leave raw areas
Nikolsky sign positive
Mucosal involvement ( eyes, genitals)
Investigations of bullous disorders
Skin biopsy with direct immunofluorescence ( half normal / half mixed)
OR
Indirect immunofluoresence ( circulating skin antibody test)
Is there mortality with bullous disorders
Particularly pemphigus if untreated
Treatment of pemphigoid and pemphigus
Systemic steroids
Immunosuppressive agents
Topical: emollients, topical steroids, topical anti sepsis / hygiene measures
In pemphigoid : tetracycline
What’s the autoantibodies in pemphigus vulgaris
IgG auto - antibodies made against desmoglein 3 ( this normally maintians desmosomal attachment_
End result is acantholysis
