BLISTERING DISORDERS Flashcards
What is bulbous pemphigoid?
An autoimmune condition causing sub-epidermal blistering of the skin
What causes bullous pemphigoid?
Autoantibodies against antigens between the epidermis and dermis (hemidesmosomal proteins) causing a sub-epidermal split in the skin
Which age group is bullous pemphigoid most common in?
Elderly patients
Presentation of bullous pemphigoid?
Itchy, tense blisters typically around the flexure
The blisters usually heal without scarring
Rare for mucosal involvment compared to pemphigus
Investigtaions for bullous pemphigoid?
Skin biopsy - immunofluorescence will show IgG and C3 at the dermoepidermal junction
Management of bullous pemphigoid?
Referral to dermatology for biopsy and confirmation of diagnosis
Oral corticosteroids
(Sometimes topical corticosteroids, immunosuppressants and antibiotic are used)
What is pemphigus vulgaris?
An autoimmune disease caused by antibodies directed against desmoglein 3, a cancer in-type epithelial cell adhesion molecule
Who is pemphigus vulgaris more common in?
the Ashkenazi Jewish population
Features of pemphigus vulgaris?
Mucosal ulceration in up to 70% and is often the presenting symptms
Skin blistering - flaccid, easily sutured vesicles and bullae. Lesions not painful but not itchy
What is nikolskys sign?
Slight rubbing of the skin results in exfoliation of the outermost layer - present in pemphigus vulgaris but not bullous pemphigoid
Biopsy findings in pemphigus vulgaris?
Acantholysis - loss of coherence between epidermal cells due to the breakdown of intercellular bridges
Management of pemphigus vulgaris?
Steroids are first line
Immunosuppressants can be used
