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Bullous pemphigoid is usually seen in elderly patients with the sudden onset of 1–2 cm tense blisters and bright red, urticarial plaques. Lesions often begin on the lower extremities and progress upward.


  1. Description of the lesion: tense bullae arising on skin that may be normal, erythematous, or urticarial (Figure 29-8).

  2. Bullous pemphigoid is an autoimmune disease primarily affecting the elderly.

  3. Autoantibodies are targeted against components of the epidermal basement membrane zone, thus triggering separation and blistering.

  4. The lesions heal without scarring.

  5. Most cases occur sporadically without obvious precipitating factors.

  6. Character and location of the lesion

    1. Predilection of blisters for the extremities

    2. Lesions range from asymptomatic to intensely pruritic.

    3. Mucosal surfaces are rarely involved.

  7. Other blistering syndromes, such as pemphigus vulgaris and epidermolysis bullosa acquisita, are caused by antibodies to other elements of the basement membrane zone.

Figure 29-8.

Bullous pemphigoid. (Used with permission from Dr. Duri Yun.)


  1. Histopathology provides supportive information, demonstrating a subepidermal blister plane and accumulation of eosinophils.

  2. Immunopathology confirms the diagnosis by demonstrating linear deposits of IgG and C3 at the dermal-epidermal junction.

  3. In 70–80% of patients, circulating IgG that recognizes the identified antigens of the basement membrane zone can be found.


  1. Topical, potent corticosteroids can be effective.

  2. Extensive disease can be treated with systemic corticosteroids.

  3. Steroid-sparing immunosuppressives are used to limit the toxicities of systemic corticosteroids in chronic disease.

  4. Alternative anti-inflammatory agents, such as tetracycline and nicotinamide, may be effective.

  5. Remission is usually obtained within a few weeks; however, some degree of long-term therapy may be necessary.

  6. Refractory cases may respond to plasmapheresis, intravenous gammaglobulin, or rituximab.

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