Bullous Pemphigoid

Chukwuemeka N. Etufugh, MD; H. Randolph Byers, MD, PhD; Tania J. Phillips, MD, FRCPC


A 75-year–old, aphasic Chinese woman presented to the authors’ wound clinic with a 6-week history of widespread blisters and erosions. Initial treatment from her primary care physician consisted of oral prednisone and topical silvadene with no improvement. The prednisone had been tapered from 80 mg to 40 mg due to a history of gastrointestinal (GI) bleeding. The patient was afebrile and had no history of fever, chills, or unexplained weight loss.
The patient’s medical history was significant for cerebrovascular accident, hypertension, upper GI bleeding, dementia, and depression. Her medications included prednisone, hydrochlorothiazide, doxazosin, metoprolol, lansoprazole (Prevacid, TAP Pharmaceuticals Inc., Deerfield, Ill), lisinopril, metoclopramide, isosorbide, aspirin, senna, and albuterol.

Physical Examination

The patient presented with well demarcated hemorrhagic tense bullae measuring 1 cm to 5 cm in diameter and multiple erosions on the trunk (Figure 1), axilla (Figure 2), back, groin (Figure 3), legs, hands, and volar aspect of the wrists, arms, and feet. Also noted were erosions on the lower lips. Lymphadenopathy was not present. The differential diagnosis included bullous pemphigoid (BP), linear IgA dermatosis, bullous drug eruption, and epidermolysis bullosa acquisita. A punch biopsy was performed on a bullous lesion of the right thigh. The biopsy revealed a mild to moderately dense superficial perivascular and interstitial mixed inflammatory cell infiltrate composed of neutrophils and eosinophils (Figure 4). Direct immunofluorescence analysis of the biopsy specimen showed linear deposition of IgG (Figure 5) and the third component of complement (C3) (Figure 6) along the basement membrane zone. Taken together, the clinical, histological, and direct immunofluorescence findings rendered a diagnosis of bullous pemphigoid.
Bullous pemphigoid is an autoimmune subepidermal blistering disease that is characterized by deposits of IgG and C3 at the epidermal basement membrane zone. Bullous pemphigoid is the most common subepidermal blistering disease found in western countries.1 It accounts for 75% of autoimmune blistering cases and is commonly found among the elderly population—66% of BP cases occur in persons 80 years or older.2,3 The annual incidence of BP has been reported to be 7 cases per million in France and Germany. Bullous pemphigoid does not have any ethnic or racial predilection and occurs equally in men and women.2,4


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