Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

ACUTE

mild pemphigus vulgaris

1st line – oral corticosteroid ± azathioprine or mycophenolate

Back
ACUTE
|
mild pemphigus vulgaris
1st line – 

oral corticosteroid ± azathioprine or mycophenolate

Prednisolone, with or without azathioprine or mycophenolate, is recommended for the initial management of mild pemphigus vulgaris. Taper corticosteroid dose according to response.[1]

Monitoring (ELISA: anti-Dsg1 and/or Dsg3 IgG) is recommended to determine the level of serum autoantibodies at the initiation of treatment.[1][29]

Primary options

prednisolone: 0.5 to 1 mg/kg/day orally

OR

prednisolone: 0.5 to 1 mg/kg/day orally

-- AND --

azathioprine: 2 mg/kg/day orally

or

mycophenolate mofetil: 1 g orally twice daily

Consider – supportive treatment

Back
ACUTE
|
mild pemphigus vulgaris
Consider – 

supportive treatment

Additional treatment recommended for SOME patients in selected patient group

Consider the following measures premised on individual patient circumstance: appropriate dental care; intralesional injection of a corticosteroid; antiseptic baths (for patients with extensive skin lesions, especially in cases of bacterial skin infection); covering erosive lesions with low adhesive wound dressings, emollients, and compresses; analgesia; nutritional management with the help of a dietician.[1][29]

Consider – bone protection

Back
ACUTE
|
mild pemphigus vulgaris
Consider – 

bone protection

Additional treatment recommended for SOME patients in selected patient group

Patients taking prolonged courses of systemic corticosteroids are at increased risk for osteoporosis and accompanying fractures. Therefore, it is imperative to monitor bone density by routine dual-energy x-ray absorptiometry (DXA) scan, and give calcium, vitamin D, and a bisphosphonate for bone supplementation.

1st line – rituximab ± oral corticosteroid

Back
ACUTE
|
mild pemphigus vulgaris
1st line – 

rituximab ± oral corticosteroid

Rituximab, with or without an adjunctive corticosteroid, is an alternative first line therapy for patients with mild pemphigus vulgaris.

Prednisolone dose may be increased to a maximum of 1 mg/kg/day in patients with persistent active lesions despite initial therapy with prednisolone plus rituximab.

Prednisolone should be tapered to a stop within 3 to 4 months in patients receiving concomitant rituximab.

Monitoring (ELISA: anti-Dsg1 and/or Dsg3 IgG) is recommended to determine the level of serum autoantibodies at the initiation of treatment.[1][29]

Primary options

rituximab: 1000 mg intravenously as a single dose on day 1 and 15

OR

rituximab: 1000 mg intravenously as a single dose on day 1 and 15

and

prednisolone: 0.5 to 1 mg/kg/day orally

Consider – supportive treatment

Back
ACUTE
|
mild pemphigus vulgaris
Consider – 

supportive treatment

Additional treatment recommended for SOME patients in selected patient group

Consider the following measures premised on individual patient circumstance: appropriate dental care; intralesional injection of a corticosteroid; antiseptic baths (for patients with extensive skin lesions, especially in cases of bacterial skin infection); covering erosive lesions with low adhesive wound dressings, emollients, and compresses; analgesia; nutritional management with the help of a dietician.[1][29]

Consider – bone protection

Back
ACUTE
|
mild pemphigus vulgaris
Consider – 

bone protection

Additional treatment recommended for SOME patients in selected patient group

Patients taking prolonged courses of systemic corticosteroids are at increased risk for osteoporosis and accompanying fractures. Therefore, it is imperative to monitor bone density by routine dual-energy x-ray absorptiometry (DXA) scan, and give calcium, vitamin D, and a bisphosphonate for bone supplementation.

2nd line – rituximab

Back
ACUTE
|
mild pemphigus vulgaris
2nd line – 

rituximab

In patients who do not achieve disease control following initial treatment with corticosteroid therapy alone (i.e., active lesions persist), rituximab is added to corticosteroid therapy.

Rituximab may also be a second line therapy for patients with corticosteroid-related adverse effects, or contraindications to azathioprine or mycophenolate.

Prednisolone should be tapered to a stop within 3 to 4 months in patients receiving concomitant rituximab.

Monitoring (ELISA: anti-Dsg1 and/or Dsg3 IgG) is recommended to determine the level of serum autoantibodies at the initiation of treatment.

Primary options

rituximab: 1000 mg intravenously as a single dose on day 1 and 15

Consider – supportive treatment

Back
ACUTE
|
mild pemphigus vulgaris
Consider – 

supportive treatment

Additional treatment recommended for SOME patients in selected patient group

Consider the following measures premised on individual patient circumstance: appropriate dental care; intralesional injection of a corticosteroid; antiseptic baths (for patients with extensive skin lesions, especially in cases of bacterial skin infection); covering erosive lesions with low adhesive wound dressings, emollients, and compresses; analgesia; nutritional management with the help of a dietician.[1][29]

mild pemphigus foliaceus

1st line – topical corticosteroid

Back
ACUTE
|
mild pemphigus foliaceus
1st line – 

topical corticosteroid

Few treatments have been evaluated in the management of pemphigus foliaceus.

A potent topical corticosteroid (e.g., betamethasone dipropionate) may be considered first line, but only in the presence of limited lesions (<5).[1]

Primary options

betamethasone dipropionate topical: (0.05%) apply sparingly to the affected area(s) once or twice daily

Consider – supportive treatment

Back
ACUTE
|
mild pemphigus foliaceus
Consider – 

supportive treatment

Additional treatment recommended for SOME patients in selected patient group

Consider the following measures premised on individual patient circumstance: appropriate dental care; intralesional injection of a corticosteroid; antiseptic baths (for patients with extensive skin lesions, especially in cases of bacterial skin infection); covering erosive lesions with low adhesive wound dressings, emollients, and compresses; analgesia; nutritional management with the help of a dietician.[1][29]

1st line – dapsone ± topical corticosteroid

Back
ACUTE
|
mild pemphigus foliaceus
1st line – 

dapsone ± topical corticosteroid

Few treatments have been evaluated in the management of pemphigus foliaceus.

Dapsone, usually combined with a potent topical corticosteroid (e.g., betamethasone dipropionate), may be considered first line.[1]

Primary options

dapsone: 50-100 mg orally once daily initially, adjust dose according to response, maximum 1.5 mg/kg/day

OR

dapsone: 50-100 mg orally once daily initially, adjust dose according to response, maximum 1.5 mg/kg/day

and

betamethasone dipropionate topical: (0.05%) apply sparingly to the affected area(s) once or twice daily

Consider – supportive treatment

Back
ACUTE
|
mild pemphigus foliaceus
Consider – 

supportive treatment

Additional treatment recommended for SOME patients in selected patient group

Consider the following measures premised on individual patient circumstance: appropriate dental care; intralesional injection of a corticosteroid; antiseptic baths (for patients with extensive skin lesions, especially in cases of bacterial skin infection); covering erosive lesions with low adhesive wound dressings, emollients, and compresses; analgesia; nutritional management with the help of a dietician.[1][29]

1st line – oral corticosteroid

Back
ACUTE
|
mild pemphigus foliaceus
1st line – 

oral corticosteroid

Few treatments have been evaluated in the management of pemphigus foliaceus.

Oral prednisolone may be considered first line.[1] 

Primary options

prednisolone: 0.5 to 1 mg/kg/day orally

Consider – supportive treatment

Back
ACUTE
|
mild pemphigus foliaceus
Consider – 

supportive treatment

Additional treatment recommended for SOME patients in selected patient group

Consider the following measures premised on individual patient circumstance: appropriate dental care; intralesional injection of a corticosteroid; antiseptic baths (for patients with extensive skin lesions, especially in cases of bacterial skin infection); covering erosive lesions with low adhesive wound dressings, emollients, and compresses; analgesia; nutritional management with the help of a dietician.[1][29]

Consider – bone protection

Back
ACUTE
|
mild pemphigus foliaceus
Consider – 

bone protection

Additional treatment recommended for SOME patients in selected patient group

Patients taking prolonged courses of systemic corticosteroids are at increased risk for osteoporosis and accompanying fractures. Therefore, it is imperative to monitor bone density by routine dual-energy x-ray absorptiometry (DXA) scan, and give calcium, vitamin D, and a bisphosphonate for bone supplementation.

1st line – rituximab ± topical or oral corticosteroid

Back
ACUTE
|
mild pemphigus foliaceus
1st line – 

rituximab ± topical or oral corticosteroid

Few treatments have been evaluated in the management of pemphigus foliaceus.

Rituximab, with or without a potent topical corticosteroid (e.g., betamethasone dipropionate) or an oral corticosteroid, may be considered first line.[1]

Prednisolone should be tapered to a stop within 3 to 4 months in patients receiving concomitant rituximab.

Primary options

rituximab: 1000 mg intravenously as a single dose on day 1 and 15

OR

rituximab: 1000 mg intravenously as a single dose on day 1 and 15

-- AND --

betamethasone dipropionate topical: (0.05%) apply sparingly to the affected area(s) once or twice daily

or

prednisolone: 0.5 mg/kg/day orally

Consider – supportive treatment

Back
ACUTE
|
mild pemphigus foliaceus
Consider – 

supportive treatment

Additional treatment recommended for SOME patients in selected patient group

Consider the following measures premised on individual patient circumstance: appropriate dental care; intralesional injection of a corticosteroid; adjuvant treatment with a super-potent topical corticosteroid (may be considered in select patients receiving rituximab alone); antiseptic baths (for patients with extensive skin lesions, especially in cases of bacterial skin infection); covering erosive lesions with low adhesive wound dressings, emollients, and compresses; analgesia; nutritional management with the help of a dietician.[1][29]

Consider – bone protection

Back
ACUTE
|
mild pemphigus foliaceus
Consider – 

bone protection

Additional treatment recommended for SOME patients in selected patient group

Patients taking prolonged courses of systemic corticosteroids are at increased risk for osteoporosis and accompanying fractures. Therefore, it is imperative to monitor bone density by routine dual-energy x-ray absorptiometry (DXA) scan, and give calcium, vitamin D, and a bisphosphonate for bone supplementation.

2nd line – rituximab ± topical or oral corticosteroid

Back
ACUTE
|
mild pemphigus foliaceus
2nd line – 

rituximab ± topical or oral corticosteroid

Consider rituximab, with or without a potent topical corticosteroid (e.g., betamethasone dipropionate) or oral corticosteroid, for patients treated initially with dapsone and/or a topical corticosteroid who have persistent active lesions, and detectable anti-DSG-1 antibodies, and significant impact on quality of life.[1]

In patients who do not achieve disease control following initial treatment with oral corticosteroid therapy (i.e., active lesions persist), rituximab is added to corticosteroid therapy.[1]

Primary options

rituximab: 1000 mg intravenously as a single dose on day 1 and 15

OR

rituximab: 1000 mg intravenously as a single dose on day 1 and 15

-- AND --

betamethasone dipropionate topical: (0.05%) apply sparingly to the affected area(s) once or twice daily

or

prednisolone: 0.5 mg/kg/day orally

Consider – supportive treatment

Back
ACUTE
|
mild pemphigus foliaceus
Consider – 

supportive treatment

Additional treatment recommended for SOME patients in selected patient group

Consider the following measures premised on individual patient circumstance: appropriate dental care; intralesional injection of a corticosteroid; adjuvant treatment with a super-potent topical corticosteroid (may be considered in select patients receiving rituximab alone); antiseptic baths (for patients with extensive skin lesions, especially in cases of bacterial skin infection); covering erosive lesions with low adhesive wound dressings, emollients, and compresses; analgesia; nutritional management with the help of a dietician.[1][29]

Consider – bone protection

Back
ACUTE
|
mild pemphigus foliaceus
Consider – 

bone protection

Additional treatment recommended for SOME patients in selected patient group

Patients taking prolonged courses of systemic corticosteroids are at increased risk for osteoporosis and accompanying fractures. Therefore, it is imperative to monitor bone density by routine dual-energy x-ray absorptiometry (DXA) scan, and give calcium, vitamin D, and a bisphosphonate for bone supplementation.

2nd line – oral corticosteroid ± azathioprine or mycophenolate

Back
ACUTE
|
mild pemphigus foliaceus
2nd line – 

oral corticosteroid ± azathioprine or mycophenolate

If rituximab is contraindicated or not available, consider prednisolone, with or without azathioprine or mycophenolate, for patients treated initially with dapsone and/or a topical corticosteroid who have persistent active lesions, and detectable anti-DSG-1 antibodies, and significant impact on quality of life.[1]

Primary options

prednisolone: 0.5 to 1 mg/kg/day orally

OR

prednisolone: 0.5 to 1 mg/kg/day orally

-- AND --

azathioprine: 1 to 2.5 mg/kg/day orally

or

mycophenolate mofetil: 1 g orally twice daily

Consider – supportive treatment

Back
ACUTE
|
mild pemphigus foliaceus
Consider – 

supportive treatment

Additional treatment recommended for SOME patients in selected patient group

Consider the following measures premised on individual patient circumstance: appropriate dental care; intralesional injection of a corticosteroid; antiseptic baths (for patients with extensive skin lesions, especially in cases of bacterial skin infection); covering erosive lesions with low adhesive wound dressings, emollients, and compresses; analgesia; nutritional management with the help of a dietician.[1][29]

Consider – bone protection

Back
ACUTE
|
mild pemphigus foliaceus
Consider – 

bone protection

Additional treatment recommended for SOME patients in selected patient group

Patients taking prolonged courses of systemic corticosteroids are at increased risk for osteoporosis and accompanying fractures. Therefore, it is imperative to monitor bone density by routine dual-energy x-ray absorptiometry (DXA) scan, and give calcium, vitamin D, and a bisphosphonate for bone supplementation.

moderate to severe pemphigus vulgaris or pemphigus foliaceus

1st line – rituximab ± oral corticosteroid

Back
ACUTE
|
moderate to severe pemphigus vulgaris or pemphigus foliaceus
1st line – 

rituximab ± oral corticosteroid

Rituximab, in association with prednisolone prescribed as a tapering dose (to stop after 6 months), is usually given first line for moderate to severe pemphigus.[1][29]

Rituximab can be administered as monotherapy if oral corticosteroid therapy is contraindicated.[1]

If there is no disease control within 1 month of initial treatment, an increased dose of prednisolone (up to 1.5 mg/kg/day) or intravenous corticosteroid pulse therapy (e.g., methylprednisolone) is recommended for patients initially treated with rituximab plus prednisolone.[1]

Monitoring (ELISA: anti-Dsg1 and/or Dsg3 IgG) is recommended to determine the level of serum autoantibodies at the initiation of treatment.[1][29]

Primary options

rituximab: 1000 mg intravenously as a single dose on day 1 and 15

-- AND --

prednisolone: 1 to 1.5 mg/kg/day orally

or

methylprednisolone sodium succinate: 0.5 to 1 g intravenously every 24 hours for 3 consecutive days at initial intervals of 3-4 weeks

More

OR

rituximab: 1000 mg intravenously as a single dose on day 1 and 15

Consider – supportive treatment

Back
ACUTE
|
moderate to severe pemphigus vulgaris or pemphigus foliaceus
Consider – 

supportive treatment

Additional treatment recommended for SOME patients in selected patient group

Consider the following measures premised on individual patient circumstance: appropriate dental care; intralesional injection of a corticosteroid; adjuvant treatment with a super-potent topical corticosteroid (may be considered in select patients receiving rituximab alone); antiseptic baths (for patients with extensive skin lesions, especially in cases of bacterial skin infection); covering erosive lesions with low adhesive wound dressings, emollients, and compresses; analgesia; nutritional management with the help of a dietician.[1][29] 

Consider – bone protection

Back
ACUTE
|
moderate to severe pemphigus vulgaris or pemphigus foliaceus
Consider – 

bone protection

Additional treatment recommended for SOME patients in selected patient group

Patients taking prolonged courses of systemic corticosteroids are at increased risk for osteoporosis and accompanying fractures. Therefore, it is imperative to monitor bone density by routine dual-energy x-ray absorptiometry (DXA) scan, and give calcium, vitamin D, and a bisphosphonate for bone supplementation.

1st line – oral corticosteroid ± azathioprine or mycophenolate

Back
ACUTE
|
moderate to severe pemphigus vulgaris or pemphigus foliaceus
1st line – 

oral corticosteroid ± azathioprine or mycophenolate

If rituximab is contraindicated or unavailable, prednisolone may be prescribed alone, or concomitantly with an immunosuppressant (azathioprine or mycophenolate), as an alternative first line therapy. Taper corticosteroid dose according to response.[1][29] 

If there is no disease control within 1 month of initial treatment, patients treated initially with prednisolone alone (and for whom rituximab cannot be prescribed) may benefit from an increased corticosteroid dose (up to 1.5 mg/kg/day), or the addition of azathioprine or mycophenolate.[1] 

Monitoring (ELISA: anti-Dsg1 and/or Dsg3 IgG) is recommended to determine the level of serum autoantibodies at the initiation of treatment.[1][29] 

Primary options

prednisolone: 1 to 1.5 mg/kg/day orally

OR

prednisolone: 1 to 1.5 mg/kg/day orally

-- AND --

azathioprine: 1 to 2.5 mg/kg/day orally

or

mycophenolate mofetil: 1 g orally twice daily

Consider – supportive treatment

Back
ACUTE
|
moderate to severe pemphigus vulgaris or pemphigus foliaceus
Consider – 

supportive treatment

Additional treatment recommended for SOME patients in selected patient group

Consider the following measures premised on individual patient circumstance: appropriate dental care; intralesional injection of a corticosteroid; antiseptic baths (for patients with extensive skin lesions, especially in cases of bacterial skin infection); covering erosive lesions with low adhesive wound dressings, emollients, and compresses; analgesia; nutritional management with the help of a dietician.[1][29]

Consider – bone protection

Back
ACUTE
|
moderate to severe pemphigus vulgaris or pemphigus foliaceus
Consider – 

bone protection

Additional treatment recommended for SOME patients in selected patient group

Patients taking prolonged courses of systemic corticosteroids are at increased risk for osteoporosis and accompanying fractures. Therefore, it is imperative to monitor bone density by routine dual-energy x-ray absorptiometry (DXA) scan, and give calcium, vitamin D, and a bisphosphonate for bone supplementation.

paraneoplastic pemphigus

1st line – referral to an oncologist

Back
ACUTE
|
paraneoplastic pemphigus
1st line – 

referral to an oncologist

For patients with paraneoplastic pemphigus (PNP), collaboration with an oncologist is a critical component of therapy (particularly before the use of rituximab is considered).[1]

PNP patients often have an active malignancy, treatment of which may benefit the PNP. However, it should be recognised that PNP can also manifest when patients are in remission.[45][46]

Therapy also involves close monitoring of lung function via pulmonary function testing as bronchiolitis obliterans and respiratory failure are often the most significant cause of mortality in patients with PNP.[32]

Monitoring (ELISA: anti-Dsg1 and/or Dsg3 IgG) is recommended to determine the level of serum autoantibodies at the initiation of treatment.[1][29]

ONGOING

moderate to severe pemphigus vulgaris or pemphigus foliaceus 6 months after initial therapy: with disease control/complete remission

1st line – continue existing therapy or rituximab

Back
ONGOING
|
moderate to severe pemphigus vulgaris or pemphigus foliaceus 6 months after initial therapy: with disease control/complete remission
1st line – 

continue existing therapy or rituximab

Patients with moderate to severe pemphigus with disease control within 1 month of treatment can continue their existing treatment (if still receiving therapy).[1]

Patients who are in complete remission, on or off therapy, may be candidates for a rituximab infusion if they initially presented with a severe pemphigus, and/or still have high levels of anti-Dsg at month 3 after initial rituximab therapy. The optimal dose of rituximab for these patients has not yet been determined.[1]

Primary options

rituximab: 500-1000 mg intravenously as a single dose

Consider – supportive treatment

Back
ONGOING
|
moderate to severe pemphigus vulgaris or pemphigus foliaceus 6 months after initial therapy: with disease control/complete remission
Consider – 

supportive treatment

Additional treatment recommended for SOME patients in selected patient group

Consider the following measures premised on individual patient circumstance: appropriate dental care; intralesional injection of a corticosteroid; adjuvant treatment with a super-potent topical corticosteroid (pending current therapy); antiseptic baths (for patients with extensive skin lesions, especially in cases of bacterial skin infection); covering erosive lesions with low adhesive wound dressings, emollients, and compresses; analgesia; nutritional management with the help of a dietician.[1][29]

Consider – bone protection

Back
ONGOING
|
moderate to severe pemphigus vulgaris or pemphigus foliaceus 6 months after initial therapy: with disease control/complete remission
Consider – 

bone protection

Additional treatment recommended for SOME patients in selected patient group

Patients taking prolonged courses of systemic corticosteroids are at increased risk for osteoporosis and accompanying fractures. Therefore, it is imperative to monitor bone density by routine dual-energy x-ray absorptiometry (DXA) scan, and give calcium, vitamin D, and a bisphosphonate for bone supplementation.

moderate to severe pemphigus vulgaris or pemphigus foliaceus 6 months after initial therapy: without complete remission

1st line – rituximab

Back
ONGOING
|
moderate to severe pemphigus vulgaris or pemphigus foliaceus 6 months after initial therapy: without complete remission
1st line – 

rituximab

Those without complete remission after 6 months (on or off therapy) can be considered for two rituximab infusions, administered 2 weeks apart.

Monitoring (ELISA: anti-Dsg1 and/or Dsg3 IgG) is recommended to determine the level of serum autoantibodies 3 months after initial treatment, and every 3 to 6 months thereafter, on the basis of evolution or relapse.[1][29]

Primary options

rituximab: 1000 mg intravenously as a single dose on day 1 and 15

Consider – supportive treatment

Back
ONGOING
|
moderate to severe pemphigus vulgaris or pemphigus foliaceus 6 months after initial therapy: without complete remission
Consider – 

supportive treatment

Additional treatment recommended for SOME patients in selected patient group

Consider the following measures premised on individual patient circumstance: appropriate dental care; intralesional injection of a corticosteroid; adjuvant treatment with a super-potent topical corticosteroid; antiseptic baths (for patients with extensive skin lesions, especially in cases of bacterial skin infection); covering erosive lesions with low adhesive wound dressings, emollients, and compresses; analgesia; nutritional management with the help of a dietician.[1][29]

moderate to severe pemphigus vulgaris or pemphigus foliaceus 12 to 18 months after initial therapy: complete remission

1st line – continue existing therapy or rituximab

Back
ONGOING
|
moderate to severe pemphigus vulgaris or pemphigus foliaceus 12 to 18 months after initial therapy: complete remission
1st line – 

continue existing therapy or rituximab

Patients with moderate to severe pemphigus with disease control within 1 month of treatment can continue their existing treatment (if still receiving therapy).[1]

In patients in complete remission (on or off therapy) with persistently high levels of anti-Dsg, one infusion of rituximab at month 12 is recommended, followed by another rituximab infusion at 18 months.[1] Patients who remain positive for anti-Dsg antibodies should be targeted. 

Additional infusions of rituximab after month 18 may be necessary for patients in whom anti-DSG antibodies recur.

Monitoring (ELISA: anti-Dsg1 and/or Dsg3 IgG) is recommended to determine the level of serum autoantibodies at the initiation of treatment.[1][29]

Primary options

rituximab: 500 mg intravenously as a single dose at month 12 and month 18

Consider – supportive treatment

Back
ONGOING
|
moderate to severe pemphigus vulgaris or pemphigus foliaceus 12 to 18 months after initial therapy: complete remission
Consider – 

supportive treatment

Additional treatment recommended for SOME patients in selected patient group

Consider the following measures premised on individual patient circumstance: appropriate dental care; intralesional injection of a corticosteroid; adjuvant treatment with a super-potent topical corticosteroid (pending current therapy); antiseptic baths (for patients with extensive skin lesions, especially in cases of bacterial skin infection); covering erosive lesions with low adhesive wound dressings, emollients, and compresses; analgesia; nutritional management with the help of a dietician.[1][29]

Consider – bone protection

Back
ONGOING
|
moderate to severe pemphigus vulgaris or pemphigus foliaceus 12 to 18 months after initial therapy: complete remission
Consider – 

bone protection

Additional treatment recommended for SOME patients in selected patient group

Patients taking prolonged courses of systemic corticosteroids are at increased risk for osteoporosis and accompanying fractures. Therefore, it is imperative to monitor bone density by routine dual-energy x-ray absorptiometry (DXA) scan, and give calcium, vitamin D, and a bisphosphonate for bone supplementation.

severe/refractory pemphigus

1st line – intravenous immunoglobulin (IVIG) or intravenous corticosteroid or immunoadsorption

Back
ONGOING
|
severe/refractory pemphigus
1st line – 

intravenous immunoglobulin (IVIG) or intravenous corticosteroid or immunoadsorption

Patients with severe/refractory disease may be recommended IVIG, intravenous corticosteroid pulse therapy (e.g., methylprednisolone), or immunoadsorption (if there is no response to rituximab treatment, or in addition to an immunosuppressant if there is no possibility to treat the patient with rituximab).[1][44]

Serum IgA deficiency should be ruled out prior to IVIG treatment; complete IgA deficiency is a contraindication for IVIG treatment. Slow IVIG infusion rates, adequate hydration, and lower doses or increased time between cycles, may reduce risk of IVIG-related adverse effects.[1][5]

Intravenous corticosteroid pulses are administered over 3 consecutive days at initial intervals of 3 to 4 weeks.[48]

Monitoring (ELISA: anti-Dsg1 and/or Dsg3 IgG) is recommended to determine the level of serum autoantibodies 3 months after initial treatment, and every 3 to 6 months thereafter, on the basis of evolution or relapse.[1][29]

Primary options

normal immunoglobulin human: 2 g/kg intravenously given over 2-5 consecutive days every 4 weeks

OR

methylprednisolone sodium succinate: 0.5 to 1 g intravenously every 24 hours for 3 consecutive days at initial intervals of 3-4 weeks

Consider – supportive treatment

Back
ONGOING
|
severe/refractory pemphigus
Consider – 

supportive treatment

Additional treatment recommended for SOME patients in selected patient group

Consider the following measures premised on individual patient circumstance: appropriate dental care; intralesional injection of a corticosteroid; adjuvant treatment with a super-potent topical corticosteroid (pending first line choice of therapy for severe/refractory pemphigus); antiseptic baths (for patients with extensive skin lesions, especially in cases of bacterial skin infection); covering erosive lesions with low adhesive wound dressings, emollients, and compresses; analgesia; nutritional management with the help of a dietician.[1][29]

Consider – bone protection

Back
ONGOING
|
severe/refractory pemphigus
Consider – 

bone protection

Additional treatment recommended for SOME patients in selected patient group

Patients taking prolonged courses of systemic corticosteroids are at increased risk for osteoporosis and accompanying fractures. Therefore, it is imperative to monitor bone density by routine dual-energy x-ray absorptiometry (DXA) scan, and give calcium, vitamin D, and a bisphosphonate for bone supplementation.

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Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer

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