PEMPHIGUS VULGARIS ================================ LISTED THERAPIES systemic: prednisone cyclophosphamide azathioprine tetracycline plus nicotinamide erythromycin plus nicotinamide cyclosporin cyclophosphamide plus vincristine and prednisone dexamethasone-cyclophosphamide pulse therapy photophoresis local for oral lesions Decadron elixer Vanceril COMMENTS FROM JERRY EISNER, M.D. [regarding a patient with oral lesions] Try Decadron elixir 0.5mg/5cc swish and swallow or swish and spit for oral lesions. Also, try Vanceril or equivalent and have patient keep spray in mouth. Add oral steroids if needed for further control. You may have reduced the steroid requirement with addition of local rx. Try type I steroids on scalp. Try tetracycline plus Nicotinamide. --------------------------------------------------- COMMENTS FROM ART HUNTLEY, M.D. My treatment of pemphigus is to begin with 80 mg/day prednisone(80 mg qam), and increase the dose to 120, then 180mg on successive weeks. At the point I believe the prednisone isn't going to be enough, I start cyclophosphamide, usually 150/day (one dose usless there is gastric upset) for a person of average weight. As an alternative I use azathioprine in the same dose. When the pemphigus is controlled I drop the prednisone rapidly to 20 mg, and then taper slowly to zero. Of course there are other variables in this equation, and sometimes the patient cannot use cyclophosphamide or azathioprine. For Pemphigus foliaceus, I have been less aggressive, perhaps because that condition doesn't seem to be as threatening to the patient as pemphigus vulgaris. -------------------------------------------- COMMENT FROM JOHN F. KAISER, M.D. With regard to your patient with pemphigus, I agree with suggestion to attempt local therapy first. If unsuccessful, I agree with use of prednisone with a steroid sparing agent. I prefer azathioprine and would start around 1 mg/kg/day (50 or 100 mg). Monitor closely for bone marrow suppression. As far as prednisone is concerned, I would also start around 1 mg/kg/day, and double dose weekly or biweekly to a maximum of 240 mg/day until adequate response achieved. It takes 2- 3 weeks for the steroid-sparing effect of azathioprine to kick-in, so do not be too hasty in tapering prednisone. I have seen significant flares of pemphigus when prednisone was tapered too rapidly. I agree that the dose of prednisone must be tapered very slowly when down in the 20- 30 mg/day range. Be prepared to treat for thrush and other forms of candidiasis. COMMENT FROM GenadijS@aol.com I reviewed J. C. Bystryn's article on "Adjuvant therapy of Pemphigus" in the Arch. Derm a few yrs. back, and spoke to him about pt. w/ P. V. His impression is to stick w/ steroids alone, esp if moderate doses work. Pt. #2 was seen in consultation at a medical center where B. Michel had spoken recently, and when I discussed Rx w/ him yesterday he told me that over the years he had observed that pts. treated w/ steroid monotherapy tended to have chronic requirements for steroids and tended to flare when tapers were attempted. On the other hand, pts. treated w/ prednisone and cytoxan from the outset, who were some of the worst at first, often obtained durable remissions after a few years. I asked him why I hadn't seen a review of these results in print, & I got the impression that he just hadn't ever compiled the data from his practice and published it. Therefore all of this remains anecdotal. I hope this triggers some pointed comments and discussion. ---------------------------------------------- COMMENT FROM MARK NAYLOR, M.D. My recommendation for limited disease: 1) Trial of tetracycline/nicotinamide 2) Topical steroids concomittantly as appropriate more serious/widespread disease: prednisone, start at 1.0-1.5 mg/kg with azathiaprine 100 mg/day started simultaneously ----------------------------------------------- COMMENT FROM K. SMITH, M.D. I have a 55 year old diabetic man whose pemphigus vulgaris did not repond adequately to prednisone up to 200 mg daily, Imuran up to 200 mg daily, Dapsone 150 mg daily niacinamide 3 gm daily and ERYC 250 tid SIMULTANEOUSLY -- but has settled down very nicely on cyclosporin, now at 100 mg daily and no prednisone for the past couple of months. The other things have been continued for the time being as cyclosporin-sparing agents. ------------------------------------------------ COMMENT FROM L. J. GREGG, M.D. In specific response to the case in question I have for some 15 years dealt with a quite similar situation in a brittle diabetic lady with the successful use of immuran as mono therapy in the 100 mg. a day range. As expected her initial response was slow but since her disease was mild (limited to facial bullae and occasional oral pharangeal lesions) this gradual onset was not a problem. The usual monitoring is required, of course. Another , similar situation in a young woman was successfully controlled for several years with clobetasol topically to oral mucous membranes and local injection of scalp lesions. In both of these cases it was striking that the lesions seem to recur in the same locale, in the first completely sparing one cheek. Overall I find initial therapy is nearly always high to moderate dose of corticosteroids with the introduction of a steroid sparing agent once control is achieved. Because of my experience (and comfort level) I prefer immuran. -------------------------------------------- COMMENT FROM JOHN MELSKI, M.D. Another consideration is IV pulse methylprednisolone, 1 gram infusion over one hour every day for three days. Our rhuematologists use pulse as an outpatient treatment in patients without cardiac rhythm problems and without risks for potassium fluxes. A baseline EKG and then a glucose and potassium determination before each infusion are prudent. An EKG rhythm strip could also be done before each infusion but is probably not necessary for otherwise healthy patients. For pemphigus, you may need to pulse as often as every two weeks but decrease to monthly as other treatments kick in. I suggest either IM gold or Imuran (azathioprine). The protocol for gold is 50 mg each week (after an initial 10 mg test dose), with a decrease in frequency after a total of 1 gram, aiming for monthly injections for maintenance. Monitoring includes watching for proteinuria. Watch out for ACE inhibitors, they can cause a pemphigus-like eruptions. Reference: Int J Dermatol 1994;33:168. When using Imuran (azathioprine), obtain a thiopurine methltransferase level. Patients with low levels of activity (.3% of the population) are at risk for severe and possibly life-threatening acute bone marrow failure. Patients with intermediate levels (11%) need to be followed closely. Reference: JAAD 1995;32:114-116. Finally, don't forget that hydroxychloroquine can be effective for pemphigus foliaceus. Reference: Arch Dermatol 1992;128:1462-4. ---------------------------------------------- SELECTED REFERENES WHICH MIGHT BE OF INTEREST Wolfe JT; Lessin SR; Singh AH; Rook AH. Review of immunomodulation by photopheresis: treatment of cutaneous T-cell lymphoma, autoimmune disease, and allograft rejection.Artificial Organs, 1994 Dec, 18(12):888-97. Luisi AF; Stoukides CA. Cyclosporine for the treatment of pemphigus vulgaris. Annals of Pharmacotherapy, 1994 Oct, 28(10):1183-5in proteinase-inhibitor system and application of proteinase inhibitors in pemphigus and pemphigoid. Journal of Dermatological Science, 1992 Sep, 4(2):95-7. Prussick R; Plott RT; Stanley JR. Recurrence of pemphigus vulgaris associated with interleukin 2 therapy [see comments]. Archives of Dermatology, 1994 Jul, 130(7):890-3. Lapidoth M; David M; Ben-Amitai D; Katzenelson V; Lustig S; Sandbank M. The efficacy of combined treatment with prednisone and cyclosporine in patients with pemphigus: preliminary study Journal of the American Academy of Dermatology, 1994 May, 30(5 Pt 1):752-7.20. Appelhans M; Bonsmann G; Orge C; Brocker EB. [Dexamethasone-cyclophosphamide pulse therapy in bullous autoimmune dermatoses]. Hautarzt, 1993 Mar, 44(3):143-7. Pandya AG; Sontheimer RD. Treatment of pemphigus vulgaris with pulse intravenous cyclophosphamide. Archives of Dermatology, 1992 Dec, 128(12):1626-30. Grando SA. Decompensation in proteinase-inhibitor system and application of proteinase inhibitors in pemphigus and pemphigoid. Journal of Dermatological Science, 1992 Sep, 4(2):95-7. Citarrella P; Gebbia V; Di Marco P; Tambone Reyes M; Noto G; Arico M. Cyclophosphamide plus vincristine and prednisone in the treatment of severe pemphigus vulgaris refractory to conventional therapy. Journal of Chemotherapy, 1992 Feb, 4(1):56-8. ------------------------------------------ (archived 25 June 1995)