Bulous Pemphigoid_sgim

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 Discussion

Learning Objectives • • • • •

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 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

Bullous Pemphigoid Associated Diseases Diabetes mellitus, rheumatoid arthritis, dermatomyositis, ulcerative colitis, and myasthenia gravis; no association with malignant conditions has been noted.

• 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.

Figure 1

Diagnosis Diagnosis relies on detecting autoantibodies bound to the skin or circulating in the serum by direct and indirect immunofluorescence microscopy, enzymelinked 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

• LABS: SODIUM: 156, K: 6.6, BUN: 124, CR: 7.6, WBC: 21.3, HB: 11.5, HCT: 37.6, PLT: 446

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.

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.

Bullous Pemphigoid and medications Furosemide (Lasix, Lo-Aqua), phenytoin (Dilantin), amoxicillin (eg, Amoxicot, Amoxil), ciprofloxacin (eg, Cipro, Ciproxin), and captopril (Capoten).

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.

Figure 3

References 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.