Bullous Disorders Flashcards
What is bullous pemphigoid?
autoimmune condition causing sub-epidermal blistering of the skin
secondary to the development of antibodies against hemidesmosomal proteins BP180 and BP230.
Bullous pemphigoid is more common in
elderly patients
Bullous pemphigois usually does/doesn’t involve the mucosa
there is usually no mucosal involvement (i.e. the mouth is spared)
Bullous pemphigoid features
itchy, tense blisters typically around flexures
the blisters usually heal without scarring
Skin biopsy in bullous pemphigoid?
immunofluorescence shows IgG and C3 at the dermoepidermal junction
Management bullous pemphidoid?
referral to a dermatologist for biopsy and confirmation of diagnosis
oral corticosteroids are the mainstay of treatment
topical corticosteroids, immunosuppressants and antibiotics are also used
What is pemphigus vulgaris?
autoimmune disease caused by antibodies directed against desmoglein 3, a cadherin-type epithelial cell adhesion molecule
Pemphigus vulgaris more common in
It is more common in the Ashkenazi Jewish population.
Pemphigus vulgaris usually does/doesn’t involve the mucosa
mucosal ulceration is common and often the presenting symptom.
Oral involvement is seen in 50-70% of patients
Pemphigus vulgaris biopsy
acantholysis on biopsy
Pemphigus vulgaris lesions?
skin blistering - flaccid, easily ruptured vesicles and bullae.
Lesions are typically painful but not itchy. These may develop months after the initial mucosal symptoms.
Nikolsky’s describes the spread of bullae following application of horizontal, tangential pressure to the skin
What is Pemphigoid gestationis?
pruritic blistering lesions
often develop in peri-umbilical region, later spreading to the trunk, back, buttocks and arms
usually presents 2nd or 3rd trimester and is rarely seen in the first pregnancy
Pemphigoid gestationis? mx
oral corticosteroids are usually required
Mx pemphigus vulgaris
steroids are first-line
immunosuppressants
