Pemphigus / Pemphigoid

Pemphigus affects approximately 0.5–3.2 per 100,000; pemphigoid affects 2.4–21.7 per 100,000·5 treatments

Pemphigus and pemphigoid are rare autoimmune blistering diseases of the skin and mucous membranes. In pemphigus, antibodies attack proteins that hold skin cells together, causing painful blisters and erosions. In pemphigoid, antibodies attack the layer between the epidermis and dermis. Both conditions can be debilitating and, without treatment, pemphigus can be life-threatening.

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Conventional first-line treatment includes systemic corticosteroids plus a steroid-sparing immunosuppressant (mycophenolate or azathioprine). Rituximab is now recommended as first-line or early second-line therapy for pemphigus (RITUX 3 trial), but insurers may still require immunosuppressant failure. IVIG is reserved for refractory cases or when other agents are contraindicated.

Must fail conventional immunosuppressants before rituximab

Very Common

Rituximab denied for pemphigoid (less evidence than for pemphigus)

Common

IVIG denied as non-first-line therapy

Common

Biopsy documentation or immunofluorescence studies not submitted

Occasional

Diagnosis not confirmed by dermatologist or dermatopathologist

Occasional

  1. 1.Include biopsy results with direct immunofluorescence confirming diagnosis
  2. 2.Document disease severity (PDAI for pemphigus, BPDAI for pemphigoid)
  3. 3.For rituximab, cite RITUX 3 trial data showing rituximab as first-line for pemphigus vulgaris
  4. 4.Document prior immunosuppressant trials and reasons for failure (infections, cytopenias, liver toxicity)
  5. 5.Include dermatologist or dermatopathologist confirmation of diagnosis

Active clinical trials that may provide treatment at no cost.

Copay cards, patient assistance programs, and foundation grants for this condition's treatments.

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Denied for Pemphigus / Pemphigoid Treatment?

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