A native of Panama was seen for extensive bullous disease that is classic for bullous pemphigoid (BP) (Figure 184-1). The presence of numerous intact bullae would make pemphigus very unlikely. The patient was treated with oral prednisone and began to respond quickly. The patient eventually had a good outcome.
A. Extensive untreated bullous pemphigoid in a Panamanian woman. B. Close-up of intact bulla and dark crusts. (Courtesy of Eric Kraus, MD.)
BP is an autoimmune blistering disease of older adults that may cause significant morbidity and a poor quality of life. The term pemphigoid refers to its similarity to the blisters seen in pemphigus. However, BP is usually less severe than pemphigus vulgaris and is not considered a life-threatening condition.
- BP is the most frequent autoimmune blistering disease of the skin (and mucosa).
- It typically affects persons older than 65 years of age but can occur at any age.
- There is no racial or gender predilection (a recent British population study, however, suggested an increased prevalence in women).1
- Its incidence may be on the rise.1
- Although it is not considered life threatening, it has been associated with an increased risk of mortality (hazard ratio [HR] 2.3, 95% confidence interval [CI] 2 to 2.7).1
- BP is a chronic autoimmune disorder of the skin.
- Immunoglobulin (Ig) G autoantibodies against BP180 antigen of the basement membrane protein are considered pathognomonic and can be found in up to 65% of patients.2
- Anti-BP230 antibodies are present in virtually all patients but are not considered pathognomonic.3
- Binding of antibodies to the basement membrane activates the complement system, leading to chemotaxis of inflammatory cells (eosinophils and mast cells), which release proteases. The subsequent degradation of hemidesmosomal proteins leads to blister formation.
- There are several morphologically distinct clinical presentations:
- Generalized bullous form is the most common (Figure 184-1, 184-2, and 184-3). Tense bullae occur on both erythematous and normal-appearing skin surfaces. The bullae usually heal without scarring.
- Localized form of BP is less common and is limited to a small area of involvement (Figure 184-2).
- Vesicular (also known as “eczematous”) form is characterized by clusters of small tense blisters with an urticarial or erythematous base.
- Other forms are less common and include vegetative (intertriginous vegetating plaques), urticarial (without any bullae), nodular (resembling prurigo nodularis), acral (bullae on palms, soles, and face in children associated with vaccination), and generalized erythroderma (exfoliative lesions with or without vesicles/bullae).
- Pemphigoid gestationis is a variant of BP that occurs during or after pregnancy. Lesions resolve after delivery, but may recur with subsequent pregnancies, or in the nonpregnant state (Chapter 76, Pemphigoid Gestationis).4
- Drug-induced BP has been reported with drugs containing sulfhydryl groups, including penicillamine, furosemide, captopril, and ...