RISK FACTORS: Genetic factors (more common in persons of Jewish or Mediterranean descent), Medications (particularly penicillamine
GENERAL MEASURES
β’ May require reverse isolation procedures
β’ Topical treatment to prevent oozing skin from adhering to bed sheets
β’ Soak active lesions to debride and remove crusts
β’ Analgesic mouth washes (see Medications)
β’ Plasmapheresis, or cyclosporine, if patient fails to
respond to an adequate trial of recommended regimens
ACTIVITY As severity of disease and medical status of patient dictates
DIET Liquid or soft for patient with mouth lesions. Regular diet when tolerated.
PATIENT EDUCATION
β’ Use of oral analgesics
β’ Teach side effects and adverse reactions of steroids
DRUG(S) OF CHOICE
. Prednisone: high doses 60-100 mg, higher if necessary. Start at 80 mg, increase by 50% every 7 days until no new blisters. Reduce to every other day dosage when possible. Taper over 6-8 months to every other day
maintenance therapy, usually continued for years.
. Consider concomitant immunosuppressants, such as azathioprine 2-3 mg/kg/day, less effective than
cyclophosphamide, but less toxic. Use with steroid, or cyclophosphamide 1-3 mg/kg/day - second best
therapy, more toxic.
. Severe cases may require combination plasmapheresis, cyclophosphamide and prednisone or immune
globulin
. Oral analgesics prn, choose one:
. Diphenhydramine (Benadryl) elixir
. Lidocaine (Xylocaine) viscous
. Dyclonine solution or lozenges
ALTERNATIVE DRUGS
β’ Gold therapy (with or without concomitant corticosteroids)
β’ Dapsone controls some cases (especially pemphigus foliaceus)
β’ Methotrexate often helpful, but too toxic due to need for high doses
β’ Chlorambucil
β’ Cyclosporine
β’ Gold
β’ Tetracycline with niacinamide (worth a trial in mild cases)
β’ Mofetil (CellCept) as concomitant therapy and rarely as a monotherapy
β’ Immune globulin (IVIG) IV in cases where adjunctive therapy not effective with prednisone, or significant side
effects from systemic corticosteroids (such as prednisone). It has been effective as monotherapy; however, it is very costly.
β’ Rituximab (Rituxan), a chimeric anti-CD 20 monoclonal antibody (MAb); a valuable treatment for refractory
pemphigus
PATIENT MONITORING
β’ Frequent visits during acute phases
β’ If immunosuppressants prescribed, monitor blood levels frequently
β’ In elderly patients, chest x-ray to rule out reactivation of old tuberculosis, test urine daily for glycosuria
POSSIBLE COMPLICATIONS
β’ Steroid complications that can lead to morbidity and mortality
β’ Inadequate nutrition and debilitation due to pain of oral lesions
β’ Sepsis/death for untreated or poorly controlled cases
EXPECTED COURSE/PROGNOSIS
β’ Chronic. Inevitably fatal if not treated.
β’ 10% fatality with vigorous treatment
β’ Ruptured bullae require weeks to heal