The Dermatology Site

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 primarily 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 bullous pemphigoid be confused?

Bullous pemphigoid must be distinguished from other bullous disorders of the skin and mucous membranes, such as: herpes simplex virus, contact dermatitis, pemphigus vulgaris, dermatitis herpetiformis, and porphyria cutanea tarda.

How is bullous pemphigoid 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 bullous pemphigoid treated?

Steroids (e.g prednisone) and immunosuppressive agents, such as methotrexate and azathioprine, are the mainstay of therapy.

What is the prognosis for bullous pemphigoid?

With treatment, the disease tends to regress over months to years. Resultant mortality is low.