Pemphigus Erythematosus Treatment & Management

Updated: Mar 02, 2018
  • Author: Rakesh Bharti, MD, MBBS; Chief Editor: William D James, MD  more...
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Treatment

Medical Care

Topical therapy

Topical corticosteroids are useful for pemphigus erythematosus patients with limited disease or as an adjunct to systemic therapy. Selection of the appropriate topical steroid strength and vehicle depends on the body site, the age of the patient, and the potential for steroid adverse effects. [8] Use of daily sunscreen and sun protection is necessary. Griffies et al reported promising results in treating discoid lupus and pemphigus erythematosus in dogs with topical application of 0.1% tacrolimus, an immunomodulator produced by a fungus. [9] Remission with tacrolimus occurred alone in some dogs, whereas steroid use was decreased or discontinued in other dogs.

Systemic therapy

Systemic steroids have been the mainstay of therapy for widespread pemphigus since their first use in 1950. Prednisone at 1-2 mg/kg/d as a single morning dose or as intravenous pulses may control the disease. Appropriate monitoring is critical.

Dapsone is effective in some patients with pemphigus erythematosus. [10, 11] Patients tend to respond relatively quickly, with improvement within several weeks. It can be a steroid-sparing drug. The possible mode of action is stabilization of lysosomal membranes and inhibition of polymorphonuclear leukocyte (PMN) toxicity. The recommended dose is 100-200 mg/d. Hemolytic jaundice may result in people with G-6-PD deficiency. Other adverse effects include agranulocytosis, leading to death, headaches, malaise, hepatitis, hypersensitivity reactions, and neuropathy. Caution is required.

Azathioprine is a potent immunosuppressive agent that has been used as a steroid-sparing agent. The usual doses are 0.5 -2.5 mg/kg/d, based on results of thiopurine methyltransferase activity. Those who are deficient are at an increased risk of bone marrow toxicity with this agent, as are patients who are taking allopurinol.

Other useful drugs in pemphigus erythematosus treatment are as follows:

  • Tetracycline and niacinamide [12]

  • Cyclophosphamide [13, 14]

  • Methotrexate [15]

  • Parenteral gold

  • Hydroxychloroquine

  • Plasmapheresis [16]

  • Mycophenolate mofetil [17]

  • Extracorporeal photochemotherapy

  • Rituximab

  • Dexamethasone-cyclophosphamide combination [18, 19, 20, 21]

Dexamethasone-cyclophosphamide combination therapy has recently been studied. In 2009, Kandan and Thappa reported good outcomes in 65 cases of pemphigus treated with dexamethasone-cyclophosphamide pulse therapy. [22]

Pasricha and Poonam reported the effects of a few modifications in the regimen in 123 patients treated with the dexamethasone-cyclophosphamide pulse/dexamethasone pulse (DCP/DP) regimen over a period of 5 years (1998-2002). The 3 modifications introduced into the regimen were: (1) an additional daily dose of oral betamethasone sufficient to control the disease activity during phase I, which was progressively tapered completely as the patient recovered; (2) use of systemic antibiotics, if the patient had skin lesions, and oral anticandidal drugs, if the patient had oral ulcers, until complete healing; and (3) insistence on thorough cleaning of the skin and scalp, with a normal soap and shampoo, and proper maintenance of oral hygiene in spite of skin/mucosal lesions. The regimen consisted of DCP/DP repeated in exactly 28-day cycles, along with cyclophosphamide at 50 mg/d, insistence on completing treatment, and avoidance of irregular pulses in all patients. [23]

Cyclophosphamide is contraindicated in patients who wish to have children. [24]

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Consultations

A dermatologist with expertise in using and in monitoring of these agents is recommended.

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Diet

Patients on long-term glucocorticoid therapy should increase their intake of calcium and vitamin D, as well as engaging in weight-bearing activity. Dual-energy x-ray absorptiometry (DEXA) scanning and bisphosphates should be discussed with a rheumatologist.

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Activity

Patients with pemphigus erythematosus should use appropriate sun-smart behaviors and protective clothing to minimize sun exposure that may exacerbate disease activity.

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Complications

The types of medications used to control severe pemphigus erythematosus may lead to serious iatrogenic disorders.

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Prevention

Sun avoidance and sun protection are recommended for pemphigus erythematosus patients.

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