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Pemphigus Vulgaris Management

Pemphigus Vulgaris Management: Suspected pemphigus vulgaris (adult) → Confirm the diagnosis (dermatology) → Before systemic treatment: baseline tests → ...

Pathway Overview

18 steps

Algorithm Steps

18 total

  1. 01Start

    Suspected pemphigus vulgaris (adult)

    Flaccid blisters or erosions of skin and mouth or other mucosa. Extensive erosions, cannot eat or drink, or sepsis: admit now under dermatology. Acute onset after a new drug, with skin pain or target lesions: treat as possible SJS/TEN (emergency).

  2. 02Action

    Confirm the diagnosis (dermatology)

    Biopsy and immunofluorescence confirm the diagnosis.

    • Biopsy of a recent blister edge: suprabasal acantholysis
    • Perilesional biopsy for direct IF: IgG and/or C3 on keratinocyte surfaces (chicken-wire pattern)
    • Serum ELISA: anti-Dsg3 (mucosal) and anti-Dsg1 (skin); indirect IF
    • Severe stomatitis or known lymphoma or leukaemia: exclude paraneoplastic pemphigus
    • Ask about thiol drugs (penicillamine, captopril): drug-induced pemphigus
  3. 03Action

    Before systemic treatment: baseline tests

    Send before the first dose. Severe disease: start prednisolone while results are pending; rituximab waits for the HBV result.

    • HBsAg, anti-HBc, HCV and HIV serology
    • Latent TB: IGRA (QuantiFERON); chest X-ray if positive
    • Strongyloides serology if born in or lived in an endemic area
    • Pregnancy test in women who could become pregnant
    • FBC, creatinine, electrolytes, LFTs, albumin, fasting glucose
    • TPMT and NUDT15 before azathioprine; IgA level before IVIG
    • Bone density and eye check before long-term steroids
    • Update inactivated vaccines, ideally 4 weeks before rituximab
  4. 04Warning

    Stop and check before immunosuppression

    Adults only. Children need a paediatric dermatology team.

    • HBsAg positive (any immunosuppression), or anti-HBc positive before rituximab: hepatology review and antiviral prophylaxis
    • Active infection or sepsis: treat it first; no rituximab
    • Pregnant: no mycophenolate or cyclophosphamide; specialist plan. Could become pregnant: contraception, until 12 months after rituximab
  5. 05Warning

    Latent infection: treat before high-dose steroids

    These infections can reactivate on steroids or rituximab.

    • Strongyloides positive, or at risk and result pending: ivermectin before high-dose steroids
    • Positive IGRA: infectious diseases plan for latent TB
    • HIV or HCV positive: co-manage with the specialist team
  6. 06Action

    Grade severity with PDAI, then follow the matching step

    Guide cut-offs: mild under 15, moderate 15-44, severe 45 or more. Cannot eat or drink counts as severe.

  7. 07Warning

    Severe (PDAI 45 or more) or cannot eat or drink: admit

    Treat as an inpatient until the blistering is controlled. Watch for sepsis: swab erosions; blood cultures if febrile.

    • Admit under dermatology: IV fluids, electrolytes, nutrition
    • Start prednisolone 1 mg/kg/day now; do not wait for rituximab
    • Give rituximab (first-line) once the HBV result is known
  8. 08Action

    Moderate (PDAI 15-44): rituximab plus a short prednisolone course

    Rituximab is first-line for moderate and severe pemphigus.

    • Rituximab plus prednisolone 0.5 mg/kg/day
    • Rituximab unavailable or contraindicated: prednisolone plus azathioprine or mycophenolate
  9. 09Action

    Mild (PDAI under 15): prednisolone, with or without a steroid-sparing agent

    Rituximab is an alternative in mild disease.

    • Prednisolone 0.5-1 mg/kg/day oral
    • Add azathioprine or mycophenolate if steroids are needed for more than 4 months
    • Alternative: rituximab 1000 mg IV, 2 doses 2 weeks apart
    • Very limited lesions only: super-potent topical steroid (clobetasol) may be enough, with close review
  10. 10Action

    Rituximab: first-line for moderate or severe; option in mild

    Only after the HBV result. Not in active infection. No live vaccines during treatment or while B cells are depleted.

    • Adult: 1000 mg IV, 2 doses 2 weeks apart
    • Before each dose: paracetamol, an antihistamine and methylprednisolone 100 mg IV
    • First infusion: slow rate; watch for infusion reactions
    • Heart disease: monitor closely; consider holding antihypertensives for 12 hours before
    • Maintenance (specialist): 500 mg IV at months 12 and 18, or retreat on relapse
    • Alternative: 375 mg/m2 IV weekly for 4 doses
  11. 11Action

    Prednisolone: dose and taper

    Adult doses. No guideline maximum: in obesity the specialist sets the dose. Use the lowest dose for the shortest time. Diabetes: monitor glucose and adjust treatment.

    • With rituximab: 0.5 mg/kg/day (moderate) or 1 mg/kg/day (severe)
    • With rituximab: taper to stop over about 3 months (moderate) or 6 months (severe)
    • Without rituximab: 0.5-1.5 mg/kg/day
    • Start the taper at disease control, at the latest at the end of consolidation
    • Relapse during taper: go back to the second-to-last dose until control, then resume the taper (specialist)
    • IV pulse steroids: refractory disease only
  12. 12Action

    Steroid-sparing agent: if no rituximab, or steroids needed long term

    Check TPMT and NUDT15 before azathioprine. Taking allopurinol or febuxostat: do not use azathioprine without specialist dose advice (severe marrow toxicity). Severe renal impairment (GFR under 25 mL/min): mycophenolate mofetil no more than 1 g twice daily.

    • Azathioprine 1-3 mg/kg/day oral (adult); start 50 mg/day for the first week
    • Low TPMT or NUDT15 activity: lower the azathioprine dose or avoid it
    • Mycophenolate mofetil 2 g/day in 2 doses (up to 3 g/day), or mycophenolic acid 1440 mg/day
    • Mycophenolate: negative pregnancy test and effective contraception first
    • Check FBC and LFTs regularly
  13. 13Action

    Supportive care: all patients

    Start with the first treatment.

    • Skin: non-adherent dressings, antiseptic baths, gentle handling
    • Mouth: soft diet, local anaesthetic gel, topical steroid to erosions, dental care
    • Pain relief; dietitian if intake is poor
    • Steroids: calcium and vitamin D; bisphosphonate if postmenopausal or a man over 50 on steroids for more than 3 months
    • Consider PJP prophylaxis (co-trimoxazole) with rituximab or prednisolone over 20 mg/day for more than 4 weeks
    • Swab and treat infection when clinically indicated; no routine antibiotics
    • VTE prophylaxis if high risk; gastric protection case by case
    • Non-live vaccines (influenza, pneumococcal, recombinant zoster); no live vaccines
  14. 14Action

    Review every 2 weeks until disease control

    Then every 1-2 weeks in consolidation, monthly while tapering, then every 3 months.

    • Disease control: no new lesions and existing lesions healing
    • End of consolidation: no new lesions for 2 weeks and about 80% healed
    • Relapse: 3 or more new lesions in a month that do not heal within 1 week
    • Anti-Dsg ELISA at start, at 3 months, then every 3-6 months
    • Watch for steroid harms: glucose, blood pressure, mood, infection, bone, eyes
  15. 15Decision

    Disease control reached and lesions healing?

  16. If Yes
    1. 16Outcome

      Responding: taper and aim for complete remission

      Complete remission off therapy: no lesions and no systemic therapy for at least 2 months.

      • Complete remission on minimal therapy (prednisolone 10 mg/day or less for 2 months): consider stopping prednisolone
      • Stop the steroid-sparing agent 6-12 months after complete remission on minimal therapy
      • Relapse is common: keep reviews every 3 months
      • Relapse: rituximab can be repeated (specialist)
    If No
    1. 17Warning

      Not responding or relapsing: refractory pemphigus (specialist)

      Find the cause before adding treatment.

      • Check adherence, dose and infection
      • Re-biopsy; exclude paraneoplastic pemphigus
      • Repeat rituximab, from 6 months after the last course
    2. 18Action

      Refractory: further options (specialist)

      Check the IgA level before IVIG; IgA deficiency needs an IgA-depleted product. IVIG with renal impairment, thrombosis risk or obesity: lowest practicable dose and infusion rate. Cyclophosphamide is teratogenic. Continue specialist review every 2 weeks until disease control.

      • IVIG 2 g/kg per cycle over 2-5 days, monthly (adult)
      • Immunoadsorption where available
      • IV pulse steroids or cyclophosphamide: last resort
      • Consider a clinical trial

Guideline Source

EADV S2K Guidelines for Pemphigus Vulgaris and Foliaceus

DOI: 10.1111/jdv.16752View Guideline

Clinical Safety Information

Clinical Decision Support — Not a Substitute for Clinical Judgment

Individual patient factors may require deviation from these recommendations.

Known Limitations

  • Adults with confirmed pemphigus vulgaris under specialist dermatology care. Children, pregnancy and paraneoplastic pemphigus need a specialist plan.
  • In Australia, rituximab for pemphigus may need hospital drug-committee approval, and IVIG needs National Blood Authority approval.
  • PDAI severity cut-offs are a guide; there are no agreed values.
  • Rituximab-era steroid doses follow the Ritux 3 trial and EADV 2020; follow local protocols.

Contraindicated Populations

Children (paediatric dermatology team)Pregnancy or breastfeeding (specialist plan)Paraneoplastic pemphigus (different disease and treatment)

Applicable Regions

AUEUUS

AU: Rituximab was not PBS-listed for pemphigus in 2022 (Somerville et al., Australas J Dermatol 2022); check hospital formulary approval. IVIG needs authorisation under the National Blood Authority criteria. Screen for Strongyloides in people from endemic areas, including northern and remote Australia.

Version 2Next review: 2027-09-30

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Frequently Asked Questions

What is the Pemphigus Vulgaris Management?

The Pemphigus Vulgaris Management is a management clinical algorithm for Dermatology. It provides a structured decision tree to guide clinical decision-making, based on EADV S2K Guidelines for Pemphigus Vulgaris and Foliaceus.

What guideline is the Pemphigus Vulgaris Management based on?

This algorithm is based on EADV S2K Guidelines for Pemphigus Vulgaris and Foliaceus (DOI: 10.1111/jdv.16752).

What are the limitations of the Pemphigus Vulgaris Management?

Known limitations include: Adults with confirmed pemphigus vulgaris under specialist dermatology care. Children, pregnancy and paraneoplastic pemphigus need a specialist plan.; In Australia, rituximab for pemphigus may need hospital drug-committee approval, and IVIG needs National Blood Authority approval.; PDAI severity cut-offs are a guide; there are no agreed values.; Rituximab-era steroid doses follow the Ritux 3 trial and EADV 2020; follow local protocols.. Individual patient factors may require deviation from these recommendations.

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