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Clinical Updates

Amit G. Pandya, MD

Update on Pemphigus Management

Amit G. Pandya, MD

Tuesday, February 07, 2006

Pemphigus continues to be one of the most difficult dermatologic disorders to treat. Patients often have recalcitrant disease, and the medications used for treatment of this blistering disorder commonly cause side effects, some of which can be life-threatening.

Many issues have to be dealt with when managing such patients. They may present with extensive erosions, inability to eat, significant pain, weight loss, malnutrition, loss of protein through the skin, secondary infection, volume depletion, prednisone-related side effects, side effects from steroid-sparing agents, anxiety, depression, frustration, and fear. Dermatologists must be prepared to address these issues or recruit colleagues in internal medicine and other specialties to help manage affected patients. Although management of pemphigus requires knowledge of general medicine, the dermatologist is still the primary physician for these patients. Unfamiliarity and discomfort with the disease often causes the internist and general practitioner to turn to the dermatologist for guidance in all aspects of care when faced with such patients.

Diagnosis and Treatment

Of course, correct diagnosis is critical in the management of pemphigus. This requires a thorough examination of the skin and mucous membranes, biopsy for routine processing as well as direct immunofluorescence testing, and often indirect immunofluorescence examination of serum as well. Once the diagnosis has been made, a plan of therapy can be developed. For patients with moderate-to severe disease, supportive care must first be addressed. Fluids, either oral or intravenous, should be initiated early on. Adequate nutrition may require oral supplementation with high protein, high calorie fluids, a duodenal feeding tube, or even parenteral nutrition. Gentle whirlpool with an antibacterial additive can be quite helpful in debridement of necrotic skin. Topical bland ointments reduce pain from lesions, especially in areas of movement, such as the hand. Dressings should be of the non-stick type, and tape should be avoided. Tube gauze can be used to cover large areas of involvement, and it is available in various sizes.

Antibiotics are often required, the selection of which should be based on adequate cultures. Gram-positive cocci, gram-negative rods, candida, and herpes virus infections may cause skin infection, particularly in body folds and the scalp. The clinician must have a high index of suspicion regarding this problem, since the patient may succumb to a disseminated infection that started in a cutaneous lesion. Pain control may require narcotic agents in patients with extensive erosions, particularly before whirlpool and dressing changes.


Mortality in patients with pemphigus has decreased dramatically over the last 50 years. Prior to the discovery of steroids, mortality was 60-90%. In the early steroid era, mortality fell to 15-45%, and now it is 5-15%. At the present time, mortality is often related to side effects from treatment rather than from the disease itself. Although we have better therapeutic modalities to treat pemphigus, the causes of improved mortality are varied and include earlier recognition and precise diagnosis of milder disease, earlier institution of therapy, improved medical and surgical therapy of the numerous complications, and more appropriate use of steroids.

The mainstay of treatment for pemphigus remains corticosteroids. Although they may cause rapid resolution of lesions, corticosteroids alone are effective in <50% of patients. They may be given intravenously in the form of prednisolone 40-80 mg 4 times daily, which is very effective in hospitalized patients. Oral prednisone 60-100 mg/day (1 mg/kg) is the most common form of corticosteroid treatment in ambulatory patients. Pulse intravenous corticosteroids can hasten resolution of blisters acutely.

One of the first large series of pemphigus treated with corticosteroids was reported by Lever et al. in 1963.1 Of the 46 patients who were followed for 11 years, there were 16 deaths, at least 6 due to treatment. Prednisone was initiated at high doses of 120-180 mg/day, and the dose was increased to 180-240 mg/day if there was no response in 5 days. The dose was maintained at a minimum of 120/day for 6-8 weeks, and then the prednisone tapered in a logarithmic fashion. The 30 surviving patients were on little or no therapy, but there were many side effects of therapy among the 46 patients, including hyperglycemia in 21, CNS changes in 16, bacterial infection in 10, candida infection in 8, vertebral fracture in 12, peptic ulcer in 6, and various other side effects. The alarming rate of side effects caused the authors to reevaluate their dosing of prednisone.

A follow-up article was published 20 years later by the same authors, in which a different regimen had been used.2 Followed over 20 years, 84 patients were given 200-400 mg/day of prednisone for severe cases and 40 mg/day of prednisone along with 100 mg/day azathioprine for milder cases. Intermediate doses of prednisone (40-200 mg/day) were discouraged. Side effects were not presented, but it was felt that the azathioprine gave a steroid-sparing effect to the patients. The effect of these articles is still felt by dermatologists worldwide, who continue to use high doses of prednisone in the treatment of pemphigus, often with the concomitant administration of azathioprine.

Treatment of pemphigus with high-dose corticosteroids given in pulse form has been used in a small group of patients.3 Nine patients were treated with monthly intravenous infusions of 1 g methylprednisolone daily for 1-5 days along with daily oral prednisone. Six others treated with oral prednisone alone did not go into remission. Four patients in the pulse group went into remission. There was a much lower mean dose of prednisone in pulse group at 1 year. The average duration of pulse therapy was 10 months. Side effects included hyperglycemia, hypertension, electrolyte disturbances, and focal infections. Use of pulse steroids early in the disease (before day 400 of prednisone) produced better outcomes. This treatment protocol may be useful in patients with recalcitrant disease.

Side Effects of Corticosteroids

Side effects of corticosteroids are well known, and dermatologists caring for patients with autoimmune blistering diseases must be familiar with them:

  • Infections are common, including bacterial, fungal, viral, and yeast infections
  • Diabetes mellitus may develop, which often requires temporary treatment with insulin
  • Osteoporosis often occurs in patients on >10 mg/day of prednisone for over a few weeks. Therefore, bone density testing and treatment with bisphosphonates may be required, along with concomitant calcium, vitamin D, and estrogen. Postmenopausal women are particularly affected with this problem
  • Aseptic necrosis of femoral head is a well-known side effect of corticosteroids, and steroid myopathy may cause profound weakness
  • Peptic ulcer disease is a common occurrence, which can be avoided by giving corticosteroids after a meal and prescribing H2 blockers, proton pump inhibitors, or sucralfate
  • One must have a high index of suspicion for gastrointestinal bleeding, which may require stool guaiac testing and serial hemoglobin measurements for detection
  • Cataracts, mood swings, and psychosis have all been reported with corticosteroids, as well as electrolyte imbalance, such as hypernatremia and hypokalemia
  • Hypertension often develops, which may require treatment with antihypertensives
  • Patients complain of a Cushingoid appearance and weight gain, as well as easy bruisability, fluid retention, and delayed wound healing. A low salt diet and diuretics are often helpful in the treatment of fluid overload
  • As these patients are often immunosuppressed, Pneumocytis pneumonia is a risk, which may require prophylaxis with dapsone of sulfa-containing antibiotics.
  • Acute withdrawal of corticosteroids can lead to symptoms of adrenal insufficiency, including weakness, fatigue, myalgias, arthralgias, and syncope as well as a flare of disease

Sublesional corticosteroid therapy of pemphigus can help localized disease and avoid increases in prednisone doses. Triamcinolone acetonide 5-10 mg/ml is usually used, with the injections carried out sublesionally, to raise a slight wheal, with the bevel of needle up. The injections are repeated every 2-4 weeks. A topical anesthetic (Hurricaine, Cetacaine) can be effective for pain control when injecting the mucous membranes.

Care of the mucous membranes is essential, yet dentists are often reluctant to perform routine maintenance because of the fear of exacerbating the disease. Routine teeth cleaning by a hygienist is helpful for gingival disease, and it may require a burst of corticosteroids (i.e. 30 mg prednisone for 3 days, beginning the day before cleaning). Gentle brushing and flossing should be performed daily. Oral candidiasis can be treated with clotrimazole troches four times daily dissolved in the mouth, or with fluconazole 150-200 mg 1-7 times per week. Patients can be instructed to mix peroxide with warm water 1:1 and swish and spit 4 times daily to remove necrotic tissue. Equal portions (i.e. 4 oz each) of antacid liquid, diphenhydramine elixir, and dexamethasone suspension can be used in a swish-and-spit mixture every 2-4 hours for pain relief. This is particularly useful before meals and at bedtime. A topical corticosteroid gel can be applied to a small piece of gauze and kept on the affected area in the mouth for 10 minutes 3 times daily. Topical corticosteroid gels can also be applied to custom-made soft vinyl trays that are inserted in the mouth. The patient bites down on the trays and holds them in the mouth for 10 minutes 3 times daily for relief of gum disease. These trays are routinely made by dentists. Inhaled and topical corticosteroids can be helpful for nasal lesions 2-3 times per day.

Treating Resistant Disease

Despite the above efforts, patients often present with resistant disease. However, one must first define resistant disease since the treatment depends on the definition. Causes of resistant disease include:

  • Ineffective therapy of acute disease
  • Inability to taper corticosteroids after controlling disease
  • Development of side effects requiring suspension of treatment or reduction of corticosteroid dose
  • Persistent, mild disease not requiring aggressive therapy

For these reasons, adjuvant treatment of pemphigus has been used for many years. Deciding which adjuvant therapy to use and when to initiate treatment will vary depending on which of these clinical scenarios exists. Unfortunately, there are many problems in the selection of adjuvant therapy, as highlighted by Bystryn et al.4 These problems include lack of controlled studies, small numbers of patients in studies, variable severity in patients with pemphigus, variable criteria in evaluating a therapeutic response, delayed effect of steroids, and better therapy for steroid-induced complications. The basic problem is that we don't have enough data demonstrating that adjuvant therapies plus corticosteroids is any better than corticosteroids alone.

Given the problem of lack of controlled trials in pemphigus, clinicians must decide which adjuvant therapy to prescribe. The following are examples of adjuvant treatments that have been reported to be effective in patients with pemphigus:

Commonly Used Agents
Sublesional and topical steroids
Mycophenolate mofetil (Cellcept)
Oral cyclophosphamide
Intravenous immunoglobulin G (IVIG)

Less Commonly Used Agents
Tetracycline and niacinamide
Intramuscular gold
Pulse steroids
Pulse cyclophosphamide

Dapsone has been reported to help patients with mild to moderate pemphigus. It is usually used for patients with controlled or mild disease, in whom one is unable to taper prednisone. Only a few case reports and small open series have been reported. One retrospective review of 20 patients found that only 25% achieved a complete response rate with dapsone and prednisone.5

Intramuscular Gold
A report of 26 patients with pemphigus treated with gold found that 62% had a response, partial or complete, without having to stop due to toxicity.6 The prednisone dose was halved within a mean of 3 months. Unfortunately, side effects from gold and the need for weekly intramuscular injections have caused it to fall out of favor in the treatment of pemphigus.

Although azathioprine is one of the most commonly used adjuvant therapies worldwide for pemphigus, there are few studies demonstrating its effectiveness. An open study in 37 patients with pemphigus treated with azathioprine was reported in 1987.7 Twenty-nine patients were available for long-term follow-up. Azathioprine 2-3 mg/kg/day was administered along with prednisone 80-200 mg/day. The prednisone dose was reduced when >50% reduction was achieved in the number of blisters. There was a 45% remission rate overall, with a 4- to 16-year follow-up period. Thirty-eight percent of the patients were free of disease but had low titer intercellular substance Ab levels on indirect immunofluorescence testing. There were few side effects from azathioprine.

Mycophenolate Mofetil
Because of side effects from azathioprine, including hepatitis and cytopenias, mycophenolate mofetil has been studied recently in the treatment of pemphigus. This agent inhibits the de novo pathway of purine synthesis in T and B cells. Most cells rely on the de novo and salvage pathways for purine biosynthesis. Lymphocytes rely on the de novo pathway more than the salvage pathway and, therefore, are the primary target. It is used systemically for prevention of renal graft rejection. An initial study of 12 patients who had relapsed during a prednisolone taper while on azathioprine was reported in 1999. Mycophenolate was given at a dose of 2 g/day along with a high dose of prednisolone (2 mg/kg/day). Eleven of 12 patients were free of disease within 2 months. Corticosteroids were reduced when induction of new blisters ceased. The median dose of prednisolone after 9 months was only 2.5 mg/day, and there was no relapse during the median 12-month follow-up period while on mycophenolate. There was mild lymphopenia in 9 and elevated LFTs in 3. Overall, it was very effective and well tolerated.

Subsequent reports have reported lower remission rates. A larger study of 42 consecutive patients, treated with 2.5-3.0 g/day of mycophenolate, 31 with pemphigus vulgaris (PV) and 11 with pemphigus foliaceus (PF) showed remission rates of 71% and 45%, respectively. The definition or remission was absence of lesions for > 4 weeks and on <12.5 mg/day of prednisone. Partial remission (1-5 skin or mucous membrane lesions and on <12.5 mg/day of prednisone) was seen in 3% of patients with PV and 36% with PF. The median time to achieve remission was 9 months, and the median duration of mycophenolate mofetil therapy was 22 months. Side effects were seen in 23% and were mostly gastrointestinal. Overall, mycophenolate has a lower incidence of side effects than azathioprine; however, the cost is much greater. It is rapidly replacing azathioprine as a first-line choice for adjuvant therapy of pemphigus; however, it may not be as effective as high-dose azathioprine (3.5-4.5 mg/kg/day).10

Tetracycline and Niacinamide
While the tetracyclines and niacinamide have been found to be effective in the treatment of bullous pemphigoid, their use in pemphigus has been less enthusiastic. This is most likely due to the fact that pemphigoid is an inflammatory blistering disease that responds to anti-inflammatory agents such as the tetracyclines. The cell-poor infiltrates of pemphigus reflect the need to treat this disease systemically, with the ultimate aim being to decrease antibody production by B cells. A small open study of 11 patients with pemphigus treated with tetracycline 2 g/day + niacinamide 1.5 g/day reported a complete response in 5 patients and a partial response in 4.11 Most were also on prednisone, which, as with all open studies reported in this paper, could have been the reason for the responses. Another study using minocycline 100 mg/d in 10 patients reported a major response in 4 and minor in 2.12 Average time to a response was 8 months. Nine of the patients were on prednisolone, and 5 on azathioprine.

Oral Cyclophosphamide
Oral cyclophosphamide, while having more side effects than mycophenolate and azathioprine, has shown good response rates in open studies. A study of 20 patients with PV, and 3 with PF (the most difficult cases in a single center) was recently reported.13 These patients had failed to achieve remission with prednisone + azathioprine or mycophenolate mofetil, had clinically significant side effects with previous therapies, or had rapidly progressive disease. Oral cyclophosphamide 2-2.5 mg/kg/day was given each morning, followed by 2-3 liters of oral fluids. The dose was adjusted to keep the absolute neutrophil count >1500 cells/mm3. Prednisone 1 mg/kg/day was also given, with tapering after 2-3 months. Nine received 4-6 plasma exchanges during the first 2 weeks for severe disease. The cyclophosphamide therapy was maintained for 9-12 months after remission. There was a complete remission in 19 patients (83%) and the median time to achieve remission was 8 months. Of the 12 who completed a course of cyclophosphamide, 6 relapsed, all with milder disease than at initial presentation. Fourteen of the 23 had adverse reactions, including nonmelanoma skin cancer, infection, GI toxicity, and microscopic hematuria, but none required discontinuation of cyclophosphamide. There was 1 case of bladder cancer, 15 years after initiating treatment. The authors concluded that cyclophosphamide may be indicated for mycophenolate mofetil and azathioprine failures.

Pulse IV Cyclophoshamide and Dexamethasone
Pulse IV cyclophosphamide and dexamethasone have been used for many patients over the years, particularly in India.14 Sixty-seven patients were treated with 100 mg/d IV dexamethasone on days 1-3 and 500 mg IV cyclophosphamide on day 1. This cycle was repeated every 4 weeks. Oral cyclophosphamide 50 mg/d was also given concomitantly. Most patients did very well. Of the 60 in remission, 50 on were on oral therapy only. At least 12 cycles of IV cyclophosphamide and dexamethasone were needed. Side effects were minimal, mainly consisting of increased susceptibility to cutaneous infections. Other articles have shown less successful results with IV cyclophosphamide in pulse doses.15

It is logical to consider removal of pathogenic antibodies in an autoimmune disease. If one can remove or reduce the amount of antibodies to epidermal antigens in the serum, the patient should improve. A prospective study compared a group of 11 patients treated with plasmapheresis, prednisone, and immunosuppressives (group 1) with 11 other patients on prednisone and immunosuppressives alone (group 2).16 All patients were hospitalized for the duration of study. Plasmapheresis was performed 3 X per week for 10-24 days. Five to 12 plasma exchanges were performed on group 1. Both groups received prednisone 40-240 mg/day. All patients in group 1 received azathioprine or cyclophosphamide 50-150 mg/day. Four of 11 patients in group 2 were treated with azathioprine 150 mg/day, and one with dapsone. There was no difference in the peak dose of prednisone required to control disease (mean 127 mg vs. 104 mg). Three weeks after admission, the ICS Ab titer had decreased 83% in group 1, but only 18% in group 2 - yet 25% of the patients in group 1 responded poorly to plasmapheresis, with no significant drop in Ab levels. Infections and hematologic complications were more common in group 1. The average dose of prednisone decreased 26.3% in group 1 and 11.1% in group 2. The authors concluded that only patients with active and extensive disease not responding to conventional therapy should receive plasmapheresis. Plasmapheresis is not commonly used in the treatment of pemphigus today.

Although initially thought to help pemphigus in several case reports, cyclosporine has now been shown to be ineffective as a steroid-sparing agent in this disease.17 A prospective, randomized trial of 33 sequential patients was carried out in Greece. All patients received steroids at a prednisone equivalent dose of 1 mg/kg/day, with an increase of 50% every 5-10 days if there was persistence of disease activity. One group also received cyclosporine 5 mg/kg/day. There was no difference between the 2 groups in all outcome measures. Complications were more common in the cyclosporine group. At this point, it appears that the combination of cyclosporine and corticosteroids offers no advantage over corticosteroids alone.

Intravenous Immunoglobulin G
One of the most recent advances in the treatment of pemphigus is the administration of intravenous immunoglobulin G (IVIG). IVIG is purified IgG from pooled plasma, and may contain some IgA as well. Anaphylaxis may occur in IgA-deficient patients, so one must measure IgA levels prior to initiating IVIG. The mechanism of action is unknown. The usual dose is 2g/kg per cycle divided over 3-5 days. A slow intravenous infusion usually prevents side effects. The cycles are repeated every 3-4 weeks, after which the frequency can be decreased as the patient improves. It should be given with caution in patients with congestive heart failure and those unable to tolerate large volumes of fluid.

A retrospective review of the literature produced information on 21 patients treated with cycles of IVIG.18 Each cycle consisted of daily infusions of immunoglobulin at approximately 650mg/kg/day for 3 consecutive days. A minimum of 2 g/kg of IVIG per cycle was given monthly for a minimum of 3 cycles. This therapy induced clinical remission in patients with recalcitrant pemphigus vulgaris. A beneficial effect was seen in 81%. The authors felt it was safe and effective. Unfortunately, IVIG has a very high cost.

Another study reported 6 patients treated with IVIG as well as prednisone 60-280 mg/day at baseline.19 IVIG was infused at a dose of 400 mg/kg/day for 5 days. Four received 1 course, 1 received 2 courses, and 1 received 3 courses. Importantly, cyclophosphamide 100-150 mg/day was also given to all patients. Improvement was rapid, and within a median of 16 days, reduction of steroid doses was possible. There was also a rapid and selective decline in circulating ICS antibodies. IVIG has been associated with pulmonary emboli, deep venous thromboses, myocardial infarctions, and other thrombotic events, as well as aseptic meningitis and acute renal failure.20,21

Rituximab is a monoclonal antibody against CD20 antigen on mature B cells. It is effective against a variety of lymphomas, and it has been used in antibody-mediated diseases as well. Three patients with severe pemphigus resistant to corticosteroids were treated with rituximab.22 Adjuvant treatments that had failed to induce remission included methotrexate, dapsone, minocycline, azathioprine, mycophenolate, IVIG, and cyclophosphamide. The duration of treatment was 1.5 to 6 years. Rituximab infusions were then given at a dose of 375 mg/m2 once weekly for 4 consecutive weeks, along with continuation of prednisone and cyclophosphamide. Two patients went into remission, and one had steady improvement. All three patients tolerated the infusions well and antibody titers fell sharply. One patient developed pneumocystis pneumonia 4 months after rituximab and died 5 weeks later. The authors recommended that rituximab should be considered in resistant cases of pemphigus, but that caution should be taken for the development of infections.


To highlight the problem with consensus agreement regarding the treatment of pemphigus, a recent telephone survey of 24 physicians worldwide with an average of 20 years of experience in treating pemphigus found that 50% see patients within 6 months after onset of symptoms and 17% within 1 year.23 The diagnosis is made by DIF in 96%. Seventy-five percent treat initially with prednisone (1 mg/kg/d in 50%, 1-1.5 mg/kg/d in 31%, and 1.5-3 mg/kg/d in 19%). Twenty-six percent of those who use prednisone initially also began adjuvants immediately. Azathioprine was used by 44%, mycophenolate by 20%, cyclophosphamide by 16%, and methotrexate by 8%. Complete discontinuation of prednisone was the goal of 37%, while the others were satisfied with doses of 2.5-10 mg/day. There is a clear need for consensus standards regarding patient stratification and design of controlled trials. Even the definitions of mild, moderate, and severe disease, as well as complete and partial remission, have not been agreed upon.

In summary, the following is a reasonable approach to adjuvant therapy for pemphigus:

  • Mild disease (<10 skin lesions or <5 mucosal lesions):
    • Intralesional/topical steroids
    • Short course of prednisone (20-40 mg/day) for less than 4 months
    • Tetracycline (or minocycline) and niacinamide
    • Dapsone
  • Moderate disease (10-30 skin lesions or 5-15 mucosal lesions) to severe disease (>30 skin lesions or >15 mucosal lesions):
    • Prednisone 60-80 mg/day (1 mg/kg/day)
    • Increase to 100 mg/d if no response in 1 week
    • Maintain until >80% of lesions healed, usually 4-12 weeks, then slow taper over 4-6 months
    • Consider alternate day steroids when dose tapered to <40 mg/day
    • For slow response, flare, or development of side effects, add:
      • Mycophenolate mofetil
      • Azathioprine
      • Oral cyclophosphamide
      • IVIG
  • Moderate-to-severe disease unresponsive to above therapies:
    • Pulse steroids
    • Plasmapheresis + immunosuppressive agent
    • Rituximab
    • Pulse cyclophosphamide
    • Chlorambucil

Finally, patient support groups can be a great source of education, comfort, empathy, and relief for affected patients. An excellent resource is the International Pemphigus Foundation (, which can provide advice and information for interested patients. It is also the umbrella organization for support groups located throughout the United States and other countries.


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