Brief Answer:
I agree with Rx & suggest that she be continued on Oral Glucocorticoids & Azathioprine

Detailed Answer:
Hello. Thank you for writing to us at healthcaremagic

I have gone through your query and I have also gone through the attachment.
I have gone through the entire sequence of events.

The involvement was initially localized to the oral mucosa and then started to involve other mucosal sites like vaginal and anal mucosa, subsequently skin as well as scalp.

It is fairly obvious that she was misdiagnosed initially as gingivitis but later diagnosed correctly as Pemphigus based on clinical features like involvement of oral mucosa, other mucosae like vaginal and anal mucosae, scalp and skin with blisters and also confirmed on biopsy.

She was started on Oral steroids and Oral azathioprine following which she started to show improvement.
The disease again made a comeback after steroid were tapered.

Pemphigus vulgaris is an autoimmune blistering disease characterized by flaccid intraepidermal fluid filled lesions/ bullae, which rupture easily and leave behind painful erosions.
The lesions commonly involve the oral mucosa; it may rather start with oral mucosal involvement. The disease may involve the scalp,extremities, torso, groin and genitals.

Treatment options are: Oral steroids/ Glucocorticoids, Oral Immunosuppressives like Cyclophosphamide, Mycophenolate Mofetil, Azathioprine etc.

I agree with the treatment line she was on i.e Oral Glucocorticoids along with Azathioprine, which is a steroid sparing agent.

The basis of giving steroids along with Azathioprine is that since daily oral steroids have a lot of adverse effects like weight gain, diabetes, high blood pressure, glaucoma, cataracts, osteoporosis etc they should not be continued alone forever, but instead patients should be simultaneously started on one of or the other safer but slow acting options (steroid sparing agents) like cyclophosphamide, Mycophenolate Mofetil, Azathioprine, OR Methotrexate.

Steroids can be tapered gradually and finally stopped once the steroids sparing agents take full control of the disease.

The disease may however relapse on tapering steroids and patients may require repeat courses of Oral steroids.
Sometimes it is not possible to taper and take the patients completely Off steroids.
Patients may relapse as soon as steroids are tapered and it is difficult to maintain patients only on steroid sparing agents like Azathioprine.
In such cases other options can be explored like DCP (Dexamethasone pulse therapy).

However, In my view she should be restarted on Daily Oral steroids as before, particularly purely glucocorticoids like methylprednisolone along with Azathioprine.
Tapering of steroid should be slow and it should not be on a defined pattern; it should be guided by the way disease behaves.
Steroids equivalent to 1-2mg/kg/day of prednisolne (i.e 60-100 mg daily or even more) usually achieve remission in 3-4 weeks(i.e no new lesions and previous lesion healing).
This improvement should be consolidated for another 2-4 weeks and if there are no new lesions and all previous lesion shave healed, steroids may be tapered by 50 % every 2-4 weeks till 20 mg daily dose is reached, after which tapering should be slow i.e by just 5 mg every 2 weeks and so on till they are completely taken off.
Tapering protocol can vary between dermatologist but the underlying fact is that it should be slow

However, if anytime during tapering new lesions appear, the dose should be hiked to the starting dose.

Azathioprine is a good choice. It takes around 4 weeks to achieve its full effects and therefore by the time the doctor considers tapering steroids, azathioprine would have reached its full effects.

Rituximab, IVIg are newer treatment options but they are expensive and can only be administered in a hospital setting.

Regards
Take care

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Brief Answer:
The dose of steroids can be hiked; MMF is a good option

Detailed Answer:
Hi.

Lack of improvement clearly indicates that she is on a lesser dose of Oral steroids and Oral immunosuppressives.
In my view her steroid dose is half of what should be given (36 mg deflazacort is equivalent to 30 mg of prednisolone/ omnacortil).
In pemphigus, steroids are usually given in a dose of 1-2 mg/kg/day Therefore the dose of deflazacort should be equivalent to 1-2 mg/kg/day of prednislone i.e if for example she weighs 60 kgs the dose of prednisone should be 60 mg daily Or 72 mg of deflazacort!!
Therefore I think she can be given a higher dose of Oral daily steroid. In my view her daily dose of steroids is just half of what it should be.

Azathioprine can be continued with this dose.

Regarding the choice between Azathioprine Or Mycophenolate Mofetil, the later can be considered.
Mycophenolate mofetil (MMF) is another immunosuppressant. It is also steroid sparing as Azathioprine but may have an inferior steroid sparing effect compared to azthioprine.
However, in pemphigus MMF is more effective as an adjuvant than azathioprine in inducing disease control.

She can go outstation if she feels. However, it is important to take regular treatment and follow up regularly with her doctor as pemphigus can recur.

Regular platelet counts (2 weekly) is required because of hematologic toxicity of immunosuppressant, both azathioprine and MMF.

Regards

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