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AT-A-GLANCE

AT-A-GLANCE

  • Bullous pemphigoid usually occurs in older adults.

  • The yearly mortality rate varies from 6% to 40%.

  • Bullous pemphigoid consists of pruritic urticarial plaques and tense, large blisters. Oral mucous membrane erosions occur in a minority of patients.

  • Skin pathology shows subepidermal blisters with eosinophils and other inflammatory cells.

  • Direct immunofluorescence (IF) shows C3 and immunoglobulin (Ig) G at epidermal basement membrane zone of perilesional skin. Indirect IF shows IgG anti–basement membrane autoantibodies in the serum.

  • The autoantigens BP180 and BP230 are proteins of the keratinocyte hemidesmosome, a basal cell–basement membrane adhesion structure.

  • Therapy includes topical and systemic corticosteroids and immunosuppressives.

INTRODUCTION

Bullous pemphigoid is the most common autoimmune blistering disorder in the adult population. The disease typically presents as pruritic, tense blisters often on a background of urticarial plaques. It occurs most frequently in older adults. It is mediated by autoantibodies directed against hemidesmosomal proteins BP180 and BP230, which trigger an inflammatory cascade that ultimately leads to blister formation. This chapter addresses the epidemiology, clinical features, pathogenesis, diagnosis, and treatment of bullous pemphigoid.

HISTORICAL PERSPECTIVE

Bullous pemphigoid was originally described as a subepidermal blistering disease with distinctive clinical and histologic features by Walter Lever in 1953.1 Antibodies against the dermal–epidermal junction were first described in perilesional skin and in the serum of patients by Jordon and Beutner 14 years later, confirming the separation of bullous pemphigoid from pemphigus.2 Over the following years, the antigenic targets were fully characterized as hemidesmosomal proteins BP180 and BP230.3-5

EPIDEMIOLOGY

Bullous pemphigoid typically occurs in patients older than 60 years of age, with a peak incidence in the 70s.6 There are exceptions in which classic bullous pemphigoid occurs in middle-aged adults and even infants and children, which is especially rare.7-10 There is no known ethnic, racial, or sexual predilection for developing bullous pemphigoid.

The incidence of bullous pemphigoid is estimated to be 7 per 1 million per year in Germany and 14 per 1 million per year in Scotland.6,11 Recent studies suggest that the incidence of bullous pemphigoid is increasing and may be as high as 22 to 24 per 1 million per year in the United States and France and 43 per 1 million per year in the United Kingdom.12-14

CLINICAL FEATURES

CUTANEOUS FINDINGS

The classic form of bullous pemphigoid is characterized by large, tense blisters arising on normal skin or on an erythematous or urticarial base (Fig. 54-1A).15,16 These lesions are most commonly found on flexural surfaces, the lower abdomen, and the thighs, although they may occur anywhere. The bullae are typically filled with serous fluid but may be hemorrhagic. The Nikolsky and Asboe-Hansen signs are negative. Eroded skin from ruptured blisters usually heals spontaneously ...

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