CICATRICIAL PEMPHIGOID ---------------------------------------------------------------- Just saw a middle age male from Germany today with a diagnosis of cicatricial pemphigoid with mostly asymptomatic eyes, ulcers of mouth, penis, perianal, and esophageal stenosis. He developed total body blisters in infancy which gradually were limited to his inguinal folds by primary school. He cannot remember being without mouth and perianal blisters. Another dermatologist had him on Prednisone for ten years and Dapsone for a short period. I sent him stat to the opthamologist. How would you all treat him? He thinks he is doing pretty well on acupuncture now. Acupuncture is the treatment which enabled him to stop prednisone! Diane Thaler -------------------- I'd get biopsies for H&E and DIF to confirm the diagnosis, then while I was waiting for the reports I'd start him on Sandimmune Neoral (cyclosporine) if he was bad enough, together with Dapsone (avlosulfone), 50 mg / day the first week, then 100 mg / day for a week, then 150 mg / day. I'd also give tetracycline 750 mg bid as an anti-inflammatory, to reduce the long-term need for cyclosporine and Dapsone. Finally, I'd have him apply clobetasol propionate ointment (Temovate, Dermovate) on gauze to the oral lesions for 10 minutes tid. I have a couple of patients with cicatricial pemphigoid who have benefitted greatly from this, and one who has been able to come off systemic medications. Let the ophthalmologist decide about a topical corticosteroid for the eyes. I don't know if something strong enough to be useful in the eye would be safe. Kevin C. Smith MD FRCPC -------------------- With all the recent discussion on azathioprine in atopic dermatitis, I am reluctant to bring up the drug again, but in some cicatricial pemphigoid patients, azathioprine has made it possible to reduce the dose of prednisone significantly. I have no idea what it will do to his need for accupuncture ! Taylor Johnson -------------------- Thanks for the idea of azothiaprine. This patient does bring up the question of acupuncture. I have had several patients who have gone on their own to acupuncturists-ones on prednisone for various medical diseases-and they are now off of prednisone. Several with various colitides. Does anyone have any idea how it might work-besides the placebo effect. Didn't some famous doc once say that acupuncture always works-if you do it on ignorant peasants. I think this has proved not to be the case. Diane Thaler -------------------- Maybe accupuncture works by stimulating the adrenal glands, so you've got more endogenous corticosteroid floating around and therefore need less prednisone. If it were MY patient I'd talk to the accupuncturist, find out exactly which meridians were being treated, then get a series of serum cortisols to see if there was any correlation with treatment. I might also get the same series on myself, my wife, and whoever else might volunteer for some accupuncture; and ideally see if the accupuncturist could do sham or at least single-blind accupuncture. This would give me some quick answers, and some idea of whether it was worth persuing further. Kevin C. Smith MD FRCPC -------------------- No it doesn't. About 10 years ago I did some study on acupuncture, including measuring serum corticosteroid on hospitalized ptts and found no significant change. The patient material was too small, so it was never published. I does work, however, but I have limited myself in only treating painful single joints in patients with psoriasis arthritis and only as a painkiller. The arthritis is treated otherwise (i.e. Methotrexate). It is also beneficial in reducing pain in leg ulcers. The mechanism is by stimulating peripheral nerves that are then sending to the spinal cord where it blocks pain input in substantia 7, lamina gelatinosa in the spinal cord. The effect can be long lasting (days) Thus it works excellent as pain-controller, but I have never tried treating regular skin diseases, maybe because I fail to see any logical explanation here. Mads Nielsen, MD -------------------- The acupuncturist who had successfully treated the colitis patients says he can do nothing for inflammation in the skin. So, when a patient of mine with eczema, and one with warts, went to him-he sent them home. Diane Thaler -------------------- Grisactin 500mg/day for three months trial. Haines Ely -------------------- Dr. K, you are correct. It is griseofulvin. Now we await Haine's explanation of the mechanism in treating cp. The midwife in Germany who delivered this patient diagnosed his infant blisters as Schelblasen. Even though he had lifelong blisters and erosions perianal, esophageal, and mouth, he was not diagnosed until he came to this country in his twenties. His espophagus was first dilated at age 17. He was born in l947, editorial comment intended. I told him that happily there is a new generation of decent and brilliant German dermatologists and physicians again. How shall I proceed with the workup. I was simply going to review the old biopsies and immunoflourescence. Diane Thaler -------------------- I see lots of CP patients who actually have Linear IgA disease. This is particularly likely in pediatric-onset patients, but even so, your pt sounds odd. Could he have has TEN or SJS which then evolved into CP (as has been reported)? Dapsone is not as good as it is cracked up to be for LIGABD or CP in our CP clinic patients and we've turned to imuran or cytoxan in many of them. Guy Webster -------------------- I have used Doxycycline and Nicotinamide in Cicatricial Pemphigoid with excellent results in one patient. Jerry Eisner -------------------- That may be all you need as long as the DIF was done well. It is probably not so important whether he is one of the few SJS progressing to CP or not, so long as you know what he has now. Repeat DIF may help if his records are not revealing enough, but beware part-time IF labs. In my exp. they screw up CP biopsies all the time. << I have used Doxycycline and Nicotinamide in Cicatricial Pemphigoid with excellent results in one patient. >> Me too, but our patient population is largely recruited at an eye hospital, so there are loads of people going blind needing big-time drugs. Guy Webster -------------------- My patient is a photographer and as far as I know (he is seeing Opthamology this week) he is not near blindness, so it looks like Doxy and Niacinamide would be a possibility. Diane Thaler -------------------- I find it interesting that an acupuncturist would feel like he/she could be successful with improving inflammation of the colon through the practice of acupunture, but not be successful at treating skin problems. I know little of acupuncture; it involves needle stimulation at certain sites on the body, and these sites or this stimulation take into account meridians that are unseen patterns that may be important in the actual physiology of acupuncture. Another alternative approach to healing is the transfer of "universal energy" to effect healing; these same meridians are of import in this technique. For skin treatments, the same chakra (or portal of entry for this universal energy) is used as that used for the treatment of lung ailments. This choice of the same chakra for both is explained by the idea that both of these organs "breathe." I have no personal experience with treating skin diseases with this technique. Hey -- is this dermchat or rxderm discussion? Sounds pretty anecdotal to me, though proponents' plans include a research facility in Thailand for the more objective study of this transfer of universal energy. Answer: perhaps further study is needed? "Western" medicine (probably an antiquated term in today's superhighway world) doesn't have all the answers... or do we? T. Keith Vaughan, M.D. -------------------- Diane, no reason I can think of except it has worked in several of my patients. I presume an antiinflammatory effect, but perhaps something more mysterious. Haines Haines Ely --------------------