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Bullous Pemphigoid
What is Bullous Pemphigoid?
Bullous pemphigoid is an autoimmune disorder that results in the
formation of bullae, most often in the groin, axillae, and intertriginous areas.
It is a disease of the elderly. Oral involvement occurs in approximately 33% of
patients. Bullae form at the subepidermal junction. This results in tense
bullae that do not exhibit a "Nikolsky sign"—(in a positive
Nikolsky sign, lateral pressure results in extension of the lesion.),
distinguishing it from pemphigus vulgaris.
With what can it be confused?
Bullous pemphigoid must be distinguished from other bullous
disorders, such as: herpes simplex virus,
contact dermatitis,
pemphigus vulgaris,
dermatitis herpetiformis, and
porphyria cutanea tarda.
How is it diagnosed?
Direct and indirect immunofluorescence is positive for IgG and
complement deposits in a linear band at the dermal-epidermal junction, at the
basement membrane zone.
How is it treated?
Steroids (e.g prednisone) and immunosuppressive agents, such as
methotrexate and azathioprine, are the mainstay of
therapy.
What is the prognosis
With treatment, the disease tends to regress over months to
years. Resultant mortality is low.
Date created 04/12/2007
Last updated
01/03/2009
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