Autoimmune bullous dermatoses
Autoimmune bullous dermatoses are a selection of autoimmune blistering diseases, including pemphigus vulgaris and bullous pemphigoid. For more information including clinical guidelines and patient information leaflets refer to British Association of Dermatologists resources.
British Association of Dermatologists
Topical or systemic corticosteroid depending on condition and severity. For topical treatment see prescribing notes.
As per specialist.
As per specialist.
As per specialist.
As per specialist.
As per specialist.
Immunosuppressant therapy, either azathioprine or mycophenolate mofetil. Treatment choice depends on condition, severity, and patient specific factors. Refer to prescribing notes for more information.
As per specialist.
As per specialist.
As per specialist, initially 500mg twice daily for 6-8 weeks; increased if no response to 1000mg twice daily and then 1500mg twice daily. Once control achieved, wean to lowest effective dose.
As per specialist, initially 500mg twice daily for 6-8 weeks; increased if no response to 1000mg twice daily and then 1500mg twice daily. Once control achieved, wean to lowest effective dose.
For the treatment of patients with moderate to severe pemphigus vulgaris.
Refer to product literature.
Prescribing Notes:
General notes
- The position of specialist treatment in this specialist dermatology pathway is not intended to guide on place in therapy. The place in therapy is directed by a dermatology specialist prescriber experienced in the management of the condition, use is in line with relevant local or national guidance.
- Depending on the severity of the condition, topical treatments may be appropriate, for formulary recommendations see pathways for Eczema, treatment with potent topical corticosteroids.
- Systemic treatments for autoimmune bullous dermatoses are initiated by specialists.
- For treatments continuing in primary care responsibility for monitoring to be agreed, refer to individual board shared care policies for more information.
- For patients with swallowing difficulties prednisolone oral solution or soluble tablets may be considered. Prednisolone tablets may be dispersed in water as an alternative to soluble tablets or oral solution preparations. This is an off-label use but more cost-effective option. Refer to local board policies on the use of unlicensed (and off-label) medicines for further guidance.
Azathioprine
- Azathioprine can be prescribed, after specialist initiation, as a steroid sparing agent in severe autoimmune bullous dermatoses. The licence status depends on the condition being treated; azathioprine is licensed for use in pemphigous vulgaris. Azathioprine is contraindicated if there is absent or very low thiopurine methyltransferase (TPMT) activity.
Mycophenolate mofetil
- Mycophenolate mofetil (off-label) can be prescribed, after specialist initiation, for patients with moderate to severe immunobullous disorders requiring steroid sparing immunosuppression.
History Notes
28/11/2024
Removal of 'Prednisolone 5mg soluble tablets' and updates to associated prescribing notes - ERFC Dec 2024.
02/07/2024
Addition of Rituximab, ERFC March 24
21/11/2023
New pathway, ERWG May 23.