The AIIMS experience

The only contraindication for pulse therapy is pregnancy or if the patient is a lactating mother and feeding her infant. This is also not an absolute contra-indication; the pulse therapy is only to be postponed till the patient has delivered her baby and stopped feeding the child. Till that time, the patient is to be maintained on a regular dose of corticosteroid just sufficient to keep the disease under control or even given a shot or two of dexamethasone alone in consultation with the obstetrician.

Patients who are unmarried or those who have not yet completed their family and want to have more children, have to be given only dexamethasone pulses (DPs) and not DCPs. Cyclophosphamide in the pulses has to be avoided because it can lead to amenorrhea or azoospermia in a significant proportion of patients. The low dose daily cyclophosphamide can be continued.

During the first few years, pulse therapy was given to only a few selected patients, especially those with severe disease however, the dosage schedule was haphazard and arbitrary as we were not sure of the total regimen. The patients were also irregular in follow up; they reported for the pulses at their own convenience and tended to stop the treatment by themselves. After about 2 years, a review revealed that several of the patients treated with the pulse therapy had not developed any relapse for long periods without any maintenance treatment, and thus we realized that pemphigus could possibly be cured.

Subsequently, we formulated an arbitrary regimen divided into 4 phases which was used to treat almost all pemphigus patients. Several patients still did not complete the course and/or received the pulses at irregular intervals. The relapse rates during follow up (recurrence of the disease after having completely recovered from the disease) were 53.8% in the patients who had received incomplete treatment and 18.2% in the patients who had received their DCPs at irregular intervals. Subsequently, we tried two modifications of the regimen. In the first modification, we increased the number of the mandatory DCPs during phase II from 6 in the original regimen to 9, and correspondingly reduced the duration of phase III from 12 months to 9 months. The relapse rate in the group who received the pulses at irregular intervals was 18.7% compared to 8% in the group who received their pulses at exactly 28 day cycles. In the second modification, we used only cyclophosphamide for the pulses during phase II of the regimen. The relapse rate in this group was 23.5%.

All patients who developed a relapse were given the second course of the DCP regimen ensuring better compliance of the regimen. There were only a few patients who were persistent defaulters and needed more than 2 courses. The relapses in all cases were mild and responded easily to the next course.

Between 1982 and 1998, 500 pemphigus patients (pemphigus vulgaris 444, pemphigus foliaceus 33, pemphigus erythematosus 18, and pemphigus vegetans 5), with an almost equal sex ratio (251 males, 249 females) were enrolled for the DCP regimen. There were 44 patients younger than 20 years, 246 patients aged 20-40 years, 190 patients aged 40-60 years and 20 patients older than 60 years. Of these, 97 patients could not complete the treatment, and 19 patients died due to a variety of causes which were mostly unrelated to the disease or its treatment, or causes that were preventable with better patient management. The remaining 384 patients recovered from the disease and are living without any disease and without any maintenance treatment. Most of them have already crossed the 5 year post-treatment follow up period.

This experience led us to conclude that pemphigus can be controlled in almost every patient and if the patient strictly follows the DCP regimen, he can be cured for the rest of his life. The treatment administered during phase II and III is necessary for ultimate cure; during phase II the most effective treatment consists of 9 DCPs taken at exactly 28 day intervals, with a daily oral dose of cyclophosphamide, followed by 9 months of daily cyclophosphamide during phase III. Compromises of any kind lead to inferior results and increase the chances of a relapse.

It is also important to remember that although the DCP regimen cures pemphigus, this does not mean that the patient has been protected from developing other diseases. The patient is as prone to develop other cutaneous or mucosal diseases as any other normal individual. Therefore subsequent lesions such as dermatophytosis, scabies, pyoderma or even lichen planus or aphthous ulcers in the mouth should not cause apprehension in the patient or the dermatologist that pemphigus has recurred. Several patients continue to develop aphthous ulcers in the mouth even during pulse therapy and this has often led some dermatologists to consider the DCP regimen as a failure in a small percentage of the patients. One should be able to distinguish aphthous ulcers from pemphigus ulcers because aphthous ulcers are far more painful, have a necrotic circular centre and an inflamed red periphery, and most ulcers heal within a few days, whereas pemphigus ulcers are far more persistent.

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