Bullous Pemphigoid

Author(s): 
Chukwuemeka N. Etufugh, MD; H. Randolph Byers, MD, PhD; Tania J. Phillips, MD, FRCPC

Presentation
 

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

References: 

1. Morrison LH, Diaz LA, Anhalt GJ. Bullous pemphigoid. In: Wojnarowska F, Briggaman RA, eds. Management of Blistering Diseases. New York, NY: Chapman and Hall Medical; 1990:63–82.
2. Bernand P, Vaillant L, Labeille B, et al. Incidence and distribution of subepidermal autoimmune bullous skin diseases in three French regions. Arch Dermatol. 1995;131(1):48–52.
3. Jung M, Kippes W, Messer G, Zillikens D, Rzany B. Increased risk of bullous pemphigoid in male and very old patients: a population based study on incidence. J Am Acad Dermatol. 1999;41(2 Pt 1):266–268.
4. Zillikens D, Wever S, Roth A, Weidenthaler-Barth B, Hashimoto T, Brocker EB. Incidence of autoimmune subepidermal blistering dermatoses in a region of central Germany. Arch Dermatol. 1995;131(8):957–958.
5. Stanley JR. Bullous pemphigoid. In: Freedberg IM, Eisen AZ, Wolff K, et al, eds. Fitzpatrick’s Dermatology in General Medicine. 5th ed. New York, NY: McGraw-Hill; 1999:666–673.
6. Cram DL, Fukuyama K. Immunohistochemistry of ultraviolet induced pemphigus and pemphigoid lesions. Arch Dermatol. 1972;106(6):819–824.
7. Thomsen K, Schmidt H. PUVA-induced bullous pemphigoid. Br J Dermatol. 1976;95(5):568–569.
8. Jordon RE, Beutner EH, Witebsky E, Blumenthal G, Hale WL, Lever WF. Basement zone antibodies in bullous pemphigoid. JAMA. 1967;200(9):751–756.
9. Mutasim DF, Takahashi Y, Labib RS, Anhalt GJ, Patel HP, Diaz LA. A pool of bullous pemphigoid antigen(s) is intracellular and associated with the basal cell cytoskeleton-hemidesmosome complex. J Invest Dermatol. 1985;84(1):47–53.
10. Diaz LA, Ratrie H, Saunders WS, et al. Isolation of a human epidermal cDNA corresponding to the 180-kD autoantigen recognized bullous pemphigoid and herpes gestationis sera. J Clin Invest. 1990;86(4):1088–1094.
11. Yancey KB, Egan CA. Pemphigoid: clinical, histologic, immunopathologic, and therapeutic considerations. JAMA. 2000;284(3):350–356.
12. Stern RS. Bullous pemphigoid therapy—think globally, act locally. N Engl J Med. 2002;346(5):364–367.
13. Roujeau J, Lok C, Bastuji-Garin S, Mhalla S, Enginger V, Bernard P. High risk of death in elderly patients with extensive bullous pemphigoid. Arch Dermatol. 1998;134(4):465–469.
14. Person JR, Rogers RS 3rd. Bullous pemphigoid responding to sulfapyridine and the sulfones. Arch Dermatol. 1977;113(5):610–615.
15. Berk MA, Lorincz AL. The treatment of bullous pemphigoid with tetracycline and niacinamide. A preliminary report. Arch Dermatol. 1986;122(6):670–674.
16. Paul MA, Jorizzo JL, Fleischer AB Jr, White WL. Low-dose methotrexate in elderly patients with bullous pemphigoid. J Am Acad Dermatol. 1994;31(4):620–635.
17. Roujeau JC, Guillaume JC, Morel P, et al. Plasma exchange in bullous pemphigoid. Lancet. 1984;2(8401):486–488.
18. Beckers RC, Brand A, Vermeer BJ, Boom BW. Adjuvant high-dose intravenous gamma globulin in the treatment of pemphigus and bullous pemphigoid: experience in six patients. Br J Dermatol. 1995;133(2):289–293.
19. Joly P, Roujeau JC, Benichou J, et al. A comparison of oral and topical corticosteroids in patients with bullous pemphigoid. N Engl J Med. 2002;346(5):321–327.



Post new comment

  • Lines and paragraphs break automatically.
  • Web page addresses and e-mail addresses turn into links automatically.
  • Use to create page breaks.

More information about formatting options

Image CAPTCHA
Enter the characters shown in the image.