What is the diagnosis and treatment for Immunobullous (autoimmune blistering) disorder?

From the Guidelines

The diagnosis and treatment of Immunobullous (autoimmune blistering) disorders, such as pemphigus vulgaris, should prioritize oral prednisolone as the first-line therapy, with a suggested starting dose of 1 mg kg-1 per day, combined with steroid-sparing immunosuppressants like azathioprine or mycophenolate mofetil, as supported by the British Association of Dermatologists' guidelines 1. The treatment approach for Immunobullous disorders involves a step-wise escalation of therapy, starting with high-potency topical corticosteroids for mild, localized disease.

• For more extensive involvement, systemic therapy is required, with first-line treatment usually consisting of oral prednisone (0.5-1.5 mg/kg/day) combined with steroid-sparing immunosuppressants like azathioprine (1-3 mg/kg/day), mycophenolate mofetil (2-3 g/day), or cyclophosphamide.
• The British Association of Dermatologists' guidelines suggest that the optimum corticosteroid dosing schedule is not known, and dosing schedules are largely empirical and based on practical experience, with a suggested starting dose of prednisolone 1 mg kg-1 per day (or equivalent) in most cases, and 0.5–1 mg kg-1 in milder cases 1.
• For refractory cases, rituximab (two 1g infusions separated by two weeks) has shown excellent efficacy, particularly in pemphigus, as reported in the British Journal of Dermatology 1.
• Intravenous immunoglobulin (2 g/kg divided over 3-5 days) may be used in severe or treatment-resistant cases, as suggested by the European Dermatology Forum consensus 1.
• Supportive care is essential, including wound care with non-adherent dressings, pain management, and monitoring for secondary infections, as these disorders require prompt diagnosis through skin biopsy with direct immunofluorescence and serum antibody testing, to improve outcomes and reduce complications like scarring and infection 1. Key considerations in the management of Immunobullous disorders include:
• The importance of early intervention to improve outcomes and reduce complications
• The need for long-term monitoring to adjust medications and minimize side effects while maintaining disease control
• The use of steroid-sparing immunosuppressants to reduce the risk of corticosteroid side-effects
• The consideration of rituximab as a third-line treatment option for pemphigus, as approved by National Health Service England 1.

From the FDA Drug Label

The safety profile of the non-U.S. -licensed rituximab in patients with PV was consistent with that observed in patients with RITUXAN-treated RA and GPA and MPA PV Study 1 (NCT00784589), a randomized, controlled, multicenter open-label study, evaluated the efficacy and safety of non-U.S. -licensed rituximab in combination with short-term prednisone compared to prednisone monotherapy in 90 patients (74 Pemphigus Vulgaris [PV] patients and 16 Pemphigus Foliaceus [PF] patients)

The diagnosis of Immunobullous (autoimmune blistering) disorder, specifically Pemphigus Vulgaris (PV), can be treated with rituximab in combination with short-term prednisone. The treatment has been evaluated in a randomized, controlled, multicenter open-label study, PV Study 1 2.

• Rituximab has been shown to be effective in treating PV, with a safety profile consistent with that observed in patients with RA and GPA and MPA.
• Short-term prednisone is used in combination with rituximab to treat PV.
• The most common adverse reactions reported in PV patients treated with rituximab include infusion-related reactions, depression, herpes simplex, and alopecia.

From the Research

Diagnosis of Immunobullous Disorder

• The diagnosis of immunobullous disorder is based on the clinical presentation, histological findings, and immunological tests 3
• The most common auto-immune bullous disorder is bullous pemphigoid (BP) 4

Treatment of Immunobullous Disorder

• The treatment of immunobullous disorder includes corticosteroids and immunosuppressant adjuvants like azathioprine and mycophenolate mofetil 5
• Ultrapotent topical corticosteroids (clobetasol propionate; 20 to 40g/day) are effective treatments for BP with fewer systemic side-effects than oral high-dose corticosteroids 4
• Systemic corticosteroids are effective but doses greater than 0.5mg/kg per day are associated with severe side-effects, including decreased survival 4
• The addition of plasma exchange or immunosuppressants (azathioprine, mycophenolate mofetil) to systemic corticosteroids has not been established 4
• Biologics like rituximab have emerged as an excellent treatment option for refractory pemphigus vulgaris 5, 6, 7
• Adjuvant immunosuppressive drugs like mycophenolate mofetil and azathioprine are widely used to minimize corticosteroid-related adverse effects 6
• Cyclophosphamide still has a place in the treatment of severe relapsing autoimmune bullous diseases 6