Pemphigus Vulgaris Treatment & Management

Updated: Jun 14, 2018
  • Author: Bassam Zeina, MD, PhD; Chief Editor: Dirk M Elston, MD  more...
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Treatment

Medical Care

The aim of treatment in pemphigus vulgaris is the same as in other autoimmune bullous diseases, which is to decrease blister formation, promote healing of blisters and erosions, and determine the minimal dose of medication necessary to control the disease process. Note the images below. Therapy must be tailored for each patient, taking into account preexisting and coexisting conditions. Patients may continue to experience mild disease activity while under optimal treatment. [38]

Erosions and healing areas on the back. Erosions and healing areas on the back.
Healing areas on the chest and abdomen. Healing areas on the chest and abdomen.

Corticosteroids have improved overall mortality, but now much of the mortality and morbidity in these patients relates to the adverse effects of therapy. Whether massive doses of steroids have any advantage over doses of 1 mg/kg/d is unclear. [39]

Immunosuppressive drugs are steroid sparing and should be considered early in the course of the disease. Epidermal growth factor may speed healing of localized lesions. [40]  Many authorities now use rituximab, the anti-CD20 antibody, as first- or second-line therapy, with evidence from 2017 suggesting better outcomes when rituximab is used as a first-line agent. [41, 42, 43, 44, 45, 46, 47, 48, 49]  

Rituximab was approved by the US Food and Drug Administration (FDA) for treatment of pemphigus vulgaris in June 2018. Approval was based on a randomized trial that compared rituximab plus oral corticosteroid treatment with corticosteroid treatment alone as first-line therapy for patients with moderate-to-severe pemphigus vulgaris. [49] At month 24, 41 (89%) of 46 patients assigned to rituximab plus short-term prednisone were in complete remission off-therapy compared with 15 (34%) of 44 assigned to prednisone alone (P<.0001).

The antitumor necrosis factor drugs sulfasalazine and pentoxifylline have been reported as effective adjunctive treatments, reducing the serum level of tumor necrosis factor and resulting in rapid clinical improvement. [50] Methotrexate has also been used. [51, 52] Dapsone has been suggested as a steroid-sparing agent in the maintenance phase of pemphigus vulgaris treatment [53, 54] ; dapsone has also been suggested as a first-line agent. [55]

Intravenous immunoglobulin therapy has been suggested as efficacious in pemphigus vulgaris treatment. [56, 57, 58, 59] Amagai et al reported on the successful use of intravenous immunoglobulin in pemphigus patients who did not fully respond to systemic steroids, [60] and Asarch et al reported its use in pediatric patients. [61]

Infliximab has proven effective in some patients with refractory disease, and photodynamic therapy has been suggested as a possible adjunctive treatment for recalcitrant ulceration. [62] Mizoribine has been used for refractory ocular manifestations. [63]  

Plasmapheresis has been used in refractory cases, usually in conjunction with cytotoxic therapy. [64]

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Consultations

Management of patients with pemphigus vulgaris requires coordination of care between the dermatologist and the patient's primary care physician.

An ophthalmologist should evaluate patients with suspected ocular involvement and those requiring prolonged high-dose steroids.

Patients with oral disease may require a dentist and/or an otolaryngologist for evaluation and care.

Patients on systemic steroids should maintain adequate vitamin D and calcium intake through diet and supplements. Patients with a history of renal calculi should not receive calcium carbonate.

Patients receiving long-term systemic corticosteroids should be evaluated by a rheumatologist within the first 30 days of treatment for osteoporosis risk assessment and consideration of a bisphosphonate for prophylaxis against osteoporosis.

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Diet

No dietary restrictions are needed, but patients with oral disease may benefit from avoiding certain foods (eg, spicy foods, tomatoes, orange juice) and hard foods that may traumatize the oral epithelium mechanically (eg, nuts, chips, hard vegetables and fruit).

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Activity

Advise patients to minimize activities that traumatize the skin and that may precipitate blistering, such as contact sports. Nontraumatic exercises, such as swimming, may be helpful. Additionally, dental plates, dental bridges, or contact lenses may precipitate or exacerbate mucosal disease.

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Complications

Secondary infection, which may be either systemic or localized to the skin, may occur because of the use of immunosuppressants and the presence of multiple erosions. Cutaneous infection delays wound healing and increases the risk of scarring.

Long-term immunosuppressant therapy may result in infections and secondary malignancies (eg, Kaposi sarcoma), owing to impaired immune surveillance.

Growth retardation has been reported in children taking systemic corticosteroids and immunosuppressants.

Bone marrow suppression has been reported in patients receiving immunosuppressants. An increased incidence of leukemia and lymphoma is reported in patients receiving prolonged immunosuppression.

Impaired immune responsiveness caused by corticosteroids and other immunosuppressive drugs may result in the rapid spread of infection. Corticosteroids suppress clinical signs of infection and may allow diseases such as septicemia or tuberculosis to reach an advanced stage before diagnosis.

Osteoporosis may occur following the use of systemic corticosteroids.

Adrenal insufficiency has been reported following prolonged use of glucocorticoids.

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