RISK FACTORS: Female, age over 60, Drug associated: furosemide, phenacetin, various
penicillins
GENERAL MEASURES
β’ Soak active lesions to debride and remove crusts
β’ Analgesic mouth washes (see Medications)
β’ Plasmapheresis can be considered
PATIENT EDUCATION
β’ Use of oral analgesics
β’ Teach side effects and adverse reactions of steroids
DRUG(S) OF CHOICE
. Initially, a 3 week intensive trial of topical class I topical steroid (eg, clobetasol propionate cream) is warranted. However, if there is no significant and promising response, then institute adjunctively one of the following systemic therapies.
. Prednisone 60-80 mg qAM. Gradually taper in several weeks to maintenance level of 20-40 mg per day. Attempt switch to alternate day treatment.
. Tetracycline 2 gm/day plus niacinamide 1.5 gm/day
. Dapsone 100-200 mg/day (check G6PD before therapy)
. Consider adjunctive drugs and infrequently as monotherapy:
. Azathioprine (Imuran) 100-150 mg to reduce prednisone maintenance therapy dosage or even as only drug to maintain control of the disease (rarely monotherapy)
. Methotrexate 10-25 mg per week as another adjunctive drug (can be monotherapy)
. Mycophenolate mofetil (CellCept) 500-1000 mg bid po (can be monotherapy)
. Topical and intralesional corticosteroids may be sufficient for patients with localized disease
. Oral analgesics prn:
. Elixir of diphenhydramine (Benadryl) for oral ulcers
. Lidocaine (Xylocaine) viscous
. Dyclonine solution
ALTERNATIVE DRUGS
β’ Cyclophosphamide (Cytoxan)
β’ Cyclosporine (Sandimmune, Neoral) for resistant cases
β’ Immune globulin IV
β’ Erythromycin
β’ Steroids IV, pulsed
PATIENT MONITORING
β’ Dapsone (GGPD, methemoglobinemia, methemoglobinuria, CBC, platelets, SGOT and alkaline phosphatase
β’ Methotrexate (liver and renal function and CBC)
β’ Mofetil (CBC and GI intolerance)
β’ Cyclosporine (blood pressure, BUN, creatinine, uric acid and magnesium)
POSSIBLE COMPLICATIONS
β’ Superimposed infection (may result in death in elderly debilitated patient)
β’ Complications of steroid therapy
β’ Rare associated malignancy
β’ Untreated severe disease can be fatal
EXPECTED COURSE/PROGNOSIS
β’ A chronic disease of unpredictable duration and frequency of relapse
β’ Old lesions heal rapidly as new lesions appear
β’ Accompanying debilitation not as great as with pemphigus