Bullous Pemphigoid (BP) In 79 Year Old Female
Manju Pillai M.D., M. Chadi Alraies M.D., Abdul Hamid Alraiyes M.D., Samer Alhindi M.D., Richard Christie M.D.
Internal Medicine Residency Program, Department of Internal Medicine
Case Western Reserve University - St. Vincent Charity Hospital – Cleveland, Ohio
Learning Objectives
• Bullous pemphigoid in elderly patients.
• Bullous pemphigoid Associated diseases and medications.
• Bullous pemphigoid is a sign not a final diagnosis.
• Bullous pemphigoid pathogenesis and management.
• Bullous pemphigoid histology and pathogenesis.
The Case
• 79 Year old African-American female with past medical history of severe
dementia, CHF, DM, CVA, and vaginal prolapse was transferred from a nursing
home with altered mental status.
• The day before admission she broke down into large bullous lesions all over
the body. No change in medications or diet in the nursing home was noted. On
admission, her medications include: furosemide 40 mg twice daily, metoprolol
25 mg twice daily, ASA 325 mg daily, glucophage 500 mg twice daily and
lisinopril 40 mg daily. nursing home nurse noticed blood in her diaper few days
before admission. She uses pessary for vaginal prolapsed and uses Premarin
ointment for a long time.
• PHYSICAL EXAMINATION: She moans and moves with stimuli with altered
mental status. Skin examination revealed many 1- to 4-cm, eroded, crusted,
and healed lesions on her arms ( Figure 1 & 2 ). These lesions had first
manifested like the tense, clear vesicles that were observed on her chest, arms
and thighs. Nikolsky’s sign was negative. No oral mucosal lesions and vitals
were stable.
• LABS: SODIUM: 156, K: 6.6, BUN: 124, CR: 7.6, WBC: 21.3, HB: 11.5, HCT:
37.6, PLT: 446
Hospital Course
• All cultures were negative, CXR: NL, no source of infection found. CT of the
brain was negative.The above abnormal labs were most likely secondary to
dehydration, treated with IVF and potassium depleting cocktail. Acute renal
failure corrected.
• Skin biopsy taken and she was started on prednisone 40 mg PO daily. Since
she continued to bleed profusely vaginally, GYN consulted to role out
malignancy. Vaginal bleeding was secondary to atrophic vaginitis . Her skin
lesions responded well to the course of steroids.
• The etiology in this case was most likely her history of DM and on furosemide.
Discussion
Bullous pemphigoid is characterized by tense, subepidermal bullae with a
predilection for the groin, axillae, and flexor surfaces. The autoimmune nature of
this disorder has been confirmed with the identification of IgG antibodies to
bullous pemphigoid antigen 1, a 230-kd protein and bullous pemphigoid antigen
2, a 180-kd molecular weight transmembrane protein, both of which are localized
to the hemidesmosome. These antigens account for 90 percent of patients with
bullous pemphigoid.
Bullous Pemphigoid Associated Diseases
Diabetes mellitus, rheumatoid arthritis, dermatomyositis, ulcerative colitis, and
myasthenia gravis; no association with malignant conditions has been noted.
Figure 1 Bullous Pemphigoid and medications
Furosemide (Lasix, Lo-Aqua), phenytoin (Dilantin), amoxicillin (eg, Amoxicot,
Amoxil), ciprofloxacin (eg, Cipro, Ciproxin), and captopril (Capoten).
Diagnosis
Diagnosis relies on detecting autoantibodies bound to the skin or circulating in
the serum by direct and indirect immunofluorescence microscopy, enzyme-
linked immunosorbent assay, or immunoblot techniques.2 Circulating
antibasement membrane IgG antibodies are detected in 60% to 80% of
patients.3 Systemic immunosuppressive agents, particularly oral
corticosteroids, are the gold standard for treating BP.3
Figure 2 Treatment
Glucocorticoids. Patients with local or minimal disease can sometimes be
controlled with topical glucocorticoids alone; patients with more extensive
lesions generally respond to systemic glucocorticoids either alone or in
combination with immunosuppressive agents.
Patients will usually respond to prednisone, 40 to 60 mg/d.
In some instances, azathioprine (1 to 2 mg/kg per day), mycophenolate
mofetil (20 to 35mg/kg per day), or cyclophosphamide (1 to 2 mg/kg per day)
are necessary adjuncts.
References
Figure 3 1. Joly P, et al. A comparison of oral and topical corticosteroids in patients with bullous pemphigoid. N Engl J Med 2002;346:321.
2. Ahmed AR. Intravenous immunoglobulin therapy for patients with bullous pemphigoid unresponsive to conventional immunosuppressive treatment. J Am Acad
Dermatol 2001;45:825.