Bullous pemphigoid is an autoimmune skin disorder causing chronic, pruritic bullous eruptions in elderly patients. Diagnosis is by skin biopsy. Corticosteroids are used initially. Most patients require long-term maintenance therapy, for which a variety of drugs can be used.

In bullous pemphigoid, antibodies are directed against the basement membrane zone of the epidermis, causing separation between the epidermis and dermis. Bullous pemphigoid must be distinguished from pemphigus vulgaris (see Bullous Diseases: Pemphigus Vulgaris; Table 1: Bullous Diseases: Distinguishing Pemphigoid From Pemphigus Vulgaris), a much more serious disease.

Table 1
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Distinguishing Pemphigoid From Pemphigus Vulgaris Disorder Appearance of Lesion Oral Involvement Itching Nikolsky's Sign Prognosis Pemphigoid Tense bullae on normal-appearing or erythematous skin In one third of patients Common Often negative Usually good; occasionally fatal in the elderly Pemphigus vulgaris Flaccid bullae of various sizes Often shearing off of skin or mucosa, leaving painful erosions Typically starts in the mouth Absent Positive Mortality ≤ 10% with treatment; higher without treatment Distinguishing Pemphigoid From Pemphigus Vulgaris Disorder Appearance of Lesion Oral Involvement Itching Nikolsky's Sign Prognosis Pemphigoid Tense bullae on normal-appearing or erythematous skin In one third of patients Common Often negative Usually good; occasionally fatal in the elderly Pemphigus vulgaris Flaccid bullae of various sizes Often shearing off of skin or mucosa, leaving painful erosions Typically starts in the mouth Absent Positive Mortality ≤ 10% with treatment; higher without treatment

Symptoms and Signs

Characteristic tense bullae develop on normal-appearing or erythematous skin, most often in flexural areas. Nikolsky's sign, in which lateral pressure on skin adjacent to a blister causes epidermal detachment, is negative. Bullous pemphigoid can manifest initially as hives with annular, dusky-red, edematous lesions, with or without peripheral vesicles. Itching is common, usually without other symptoms. Oral lesions occur in about one third of patients but heal rapidly.

Bullous Pemphigoid

Diagnosis

• Skin biopsy and antibody titers

Patients should have a skin biopsy and serum antibody titers for hemidesmosomal BP antigens BP230 (BPAg1) and BP180 (BPAg2).

Bullous pemphigoid must be differentiated from pemphigus vulgaris, linear IgA disease, erythema multiforme, drug-induced eruptions, benign mucous membrane pemphigoid, paraneoplastic pemphigoid, dermatitis herpetiformis, and epidermolysis bullosa acquisita.

Prognosis

Prognosis is good, and the disorder usually subsides within months to years; however, the disorder is potentially fatal, especially in the elderly and debilitated patients, with death being caused by infection and sepsis or the effects of the drugs.

Treatment

• Corticosteroids, topical or oral
• Anti-inflammatory drugs

Mild bullous pemphigoid sometimes resolves without treatment, but resolution usually takes months or years. Patients with more severe disease receive prednisone 60 to 80 mg po once/day, which can be tapered to a maintenance level of ≤ 10 to 20 mg/day after several weeks. Most patients achieve remission after 2 to 10 mo. Occasional new lesions in elderly patients do not require increasing the prednisone dosage.

The disorder occasionally responds to a combination of tetracycline or minocycline and nicotinamide. Other treatment options include dapsone, sulfapyridine, erythromycin, and tetracycline used alone for their anti-inflammatory rather than their antibiotic properties. IV immune globulin has been used occasionally. For patients with generalized and recalcitrant disease, immunosuppressants such as azathioprine, cyclophosphamide, rituximab, and cyclosporine may be used. However, use of immunosuppressants for bullous pemphigoid is controversial.

Last full review/revision September 2008 by Julie E. Russak, MD