CHAPTER 12 AUTOIMMUNE BLISTERING DISEASES 197
Additional treatment similar to PF/PE in recalcitrant cases.
Corticosteroid monotherapy not recommended.
Many cases are chronic and mild, necessitating minimal intervention.
Drugs of Choice – Epidermolysis Bullosa Acquisita
Corticosteroids:
Prednisolone: 2.2–6.6 mg/kg/day PO divided BID to achieve clinical remission
Minimum maintenance: 0.5 mg/kg q48–72h
Taper dosage at 2–4-week intervals.
Cytotoxic agents:
Azathioprine (2 mg/kg or 50 mg/m^2 PO q24h, then q48–72h)
Mycophenolate mofetil: 20–40 mg/kg/day PO divided BID to TID
Colchicine 0.03 mg/kg PO q24h.
Cyclosporine:
Alternative or supplemental therapy with corticosteroids
Initial dosage 5 mg/kg PO daily.
Alternative Corticosteroids
Use instead of prednisolone if undesirable side effects or poor response occur.
Methylprednisolone (initial dosage 0.8–1.5 mg/kg PO BID): patients that tolerate
prednisolone poorly.
Triamcinolone (0.2–0.3 mg/kg PO BID; then 0.05–0.1 mg/kg EOD to twice weekly).
Dexamethasone (0.1–0.2 mg/kg PO q24h; then 0.05 mg/kg twice weekly).
Topical Steroids
Hydrocortisone cream.
More potent topical corticosteroids: 0.1% betamethasone valerate, fluocinolone ace-
tonide, or 0.1% triamcinonide; BID tapering to EOD or twice weekly.
COMMENTS
Patient Monitoring
Monitor response to therapy at 2–4-week intervals initially; monitor less frequently
as lesions heal and medication dosages are reduced.
Routine hematology and serum biochemistry, especially patients on high doses of
corticosteroids, cytotoxic drugs, or chrysotherapy; check every 2–4 weeks, and then
every 1–3 months when in remission. Common side effects:
Corticosteroids: polyuria, polydipsia, polyphagia, temperament changes, dia-
betes mellitus, pancreatitis, and hepatotoxicity
Azathioprine: pancreatitis, bone marrow suppression