Pemphigus Foliaceus Treatment & Management

Updated: Feb 13, 2017
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD  more...
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Treatment

Medical Care

Present information is probably inadequate to ascertain the optimal therapy for pemphigus foliaceus (PF), including the optimal glucocorticoid dose, the role of adjuvant immunosuppressive medications, and long-term adverse events to improve the risk-to-benefit ratio. [44] Therapy for pemphigus foliaceus is usually less aggressive than that of PV because of lower morbidity and mortality rates. [45]

First results indicate that nonsteroidal treatment of pemphigus is possible. Mestinon may be used to slow down progression of the disease and to treat mild cases with chronic lesions on limited areas. Antimalarial therapy may be effective monotherapy in some patients. However, a major obstacle in comparing therapeutic outcomes is the lack of generally accepted definitions and measurements for the clinical evaluation of patients with pemphigus. [46] Common terms and endpoints of pemphigus are needed to accurately measure and assess disease extent, activity, severity, and therapeutic response.

Topical glucocorticosteroids may be sufficient in cases of limited involvement. [47]

In more extensive cases (similar to PV), adjuvant immunosuppressants, including systemic corticosteroids, azathioprine, mycophenolate mofetil, cyclophosphamide, and cyclosporin A, may be necessary. [48, 49]

In some cases, such as PE, combined therapy is beneficial with the use of corticosteroids and sulfones or antimalarial agents.

Topical treatment with antibiotics and corticosteroids, such as topical clobetasol cream or ointment 0.05% twice a day, is helpful. Other vehicles that may be useful are creams, foams, liquids (for scalp lesions), and aerosols. Antibiotics, such as minocycline 50 mg daily, may be effective. Nicotinamide 1.5 g/d and tetracycline 2 g/d have also been reported to be beneficial in a small number of patients. Antibiotics and nicotinamide are purported to have anti-inflammatory effects. [50]

Photoprotection is appropriate for some patients because UV-B may trigger acantholysis and cause a flare-up of the disease.

Successful anti-CD20 antibody treatment has also been described. [51]

Plasmapheresis is another therapeutic option in patients with recalcitrant disease. It may decrease autoantibody titers in some patients and favorably influence the clinical outcome, especially in patients with otherwise therapy-resistant pemphigus foliaceus. It is often used in conjunction with cytostatic agents, such as cyclophosphamide or azathioprine, to reduce a predictable rebound increase in autoantibody synthesis. Potential complications, including the need for maintaining venous access, a bleeding tendency, electrolyte shifts, pulmonary edema, fever, chills, hypotension, and septicemia, should be considered.

Amagai et al reported that a single cycle of intravenous immunoglobulin at 400 mg/kg/d for 5 days is effective and safe for patients with pemphigus that is relatively resistant to systemic steroid therapy. [52] Toth and Jonkman also reported on successful therapy with intravenous immunoglobulin (low dose). [53]

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Complications

Monitor pemphigus foliaceus (PF) patients for other autoimmune disorders, particularly thymoma and myasthenia gravis.

New cutaneous pain or new constitutional symptoms, change in primary morphology, rapid disease progression, or failure to respond to appropriate therapies may suggest a concurrent cutaneous viral infection such as by herpes simplex or cytomegalovirus. [54]

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