|Year : 1997 | Volume
| Issue : 6 | Page : 354-356
Dexamethasone - Cyclophosphamide pulse (DCP) therapy in Pemphigus
Renu Roy, G Kalla
Source of Support: None, Conflict of Interest: None
Of the 37 patients enrolled for this treatment, 13 have been lost to follow up and 4 have died. Of the remaining 20, 8 are already in remission (40%), while 12 are still having active disease. Of the 8 patients who are now in remission, 1 is in phase II and taking monthly dexamethasone cyclophosphamide pulse and 50 mg cyclophosphamide daily while in 3 patients all the treatment has already been withdrawn (phase IV). All these patients are being followed up for any recurrence. The duration of remission has been more than 6 months in 7 patients (maximum 2 years). The chief side-effect observed was increased susceptibility to pyogenic and candidal infections of the skin and oral mucosa respectively. The other side-effects noted were generalized weakness and lethargy following DCP (1), irregular menstrual periods (1), amenorrhoea (1), general darkening of complexion (1), steroid psychosis (1), transient eosinophilia (1) and marked transient oligospermia.
Keywords: Pemphigus, Dexamethasone, Cyclophosphamide
|How to cite this article:|
Roy R, Kalla G. Dexamethasone - Cyclophosphamide pulse (DCP) therapy in Pemphigus. Indian J Dermatol Venereol Leprol 1997;63:354-6
|How to cite this URL:|
Roy R, Kalla G. Dexamethasone - Cyclophosphamide pulse (DCP) therapy in Pemphigus. Indian J Dermatol Venereol Leprol [serial online] 1997 [cited 2020 Jan 18];63:354-6. Available from: http://www.ijdvl.com/text.asp?1997/63/6/354/4616
Use of systemic corticosteroids has transformed an almost invariably fatal illness into one in which mortality is now below 10%. Most patients who die of pemphigus at present die of complications of therapy. This has led to a continued search for alternative treatments that might reduce the need for steroids and in turn morbidity/ mortality. The recent report by Pasricha et al,  of successful treatment with DCP therapy of pemphigus patients is very promising and encouraged us to undertake this study.
| Materials and Methods|| |
The diagnosis of pemphigus in each case was based on clinical characteristics and Tzanck smears from the base of blisters and histopathology of skin/mucous membrane lesions. Immunofluorescence studies were not undertaken due to lack of facilities. Laboratory evaluation that included hemoglobin, total and differential leucocyte count platelet counts, ESR, blood glucose, blood urea, SGOT, SGPT, serum electrolytes, urinalysis, stool examination for occult blood, EGG and chest x-ray, were undertaken before starting treatment and during follow-up. The treatment schedule consisted of giving 100mg dexamethasone, dissolved in 500ml of 5% dextrose, by a slow intravenous infusion over 1 ½ hours on 3 consecutive days, along with 500mg cyclophosphamide in the same infusion on day one. These pulses were repeated at 4 weekly 'intervals. In between the dexamethasone, cyclophosphamide pulses (DCP), the patients received cyclophosphamide, 50 gm orally per day. The entire treatment was divided into 4 phases. During the first phase, the lesions would heal very quickly following DCP, but after a variable number of days, there would be a recurrence of the lesions. During the next phase, the patients would remain in complete remission but one monthly DCP and daily cyclophosphamide were continued for a minimum period of 6 months (phase II). After this, the DCPs were stopped but patient continued to receive 50 mg cyclophosphamide a day orally for the next 6 months (phase III). In phase IV, even this treatment was withdrawn and the patient was followed up for any recurrence.
| Results|| |
Between January 1993 and September 1996, 37 patients of pemphigus, were treated with this regimen. The 37 patients included pemphigus vulgaris (26 cases), pemphigus foliaceus (9) and pemphigus vegetans (2). There were 15 males and 22 females. Their age ranged between 10 and 58 years when they were enrolled for DCP. The duration of the disease before treatment was 5-10 years in 3, 2-5 years in 3, 1-2 years in 6,6 months to 1 year in 6 and less than 6 months in 19 cases. Twelve patients were treated during their very first episode of pemphigus. Fifteen patients had already been on corticosteroids in their usual daily regimens and had the well known side-effects. Thirteen patients have been lost to follow-up and 4 (10.8%) have died (septicemia - 2, peripheral circulatory failure-1 and cardiac arrest - 1). Of the remaining 20 patients, 8 are already in remission, while 12 are still having active disease. Of the 8 patients in remission, 1 is in phase II, 4 in phase III and 3 in phase IV. The duration of remission has been more than 6 months in 7 patients (maximum 2 years). The duration of phase I varied widely in different patients and showed no correlation with the age or sex of the patient, with the clinical severity of the disease or its duration before starting treatment. Neither was there any correlation between the type of pemphigus and the response to therapy.
Healing of the skin lesions was much faster, lesions healing in3-4 day time. The side-effects usually associated with prolonged treatment with corticosteroids and cyclophosphamide were virtually absent. The chief side-effects shown were increased susceptibility to infections, both bacterial and candidal in almost all patients, necessitating frequent course of antibiotics and antifungals. The other side effects noted were generalized weakness and lethargy following DCP (1), irregular menstrual periods (1) amenorrhoea (1), general darkening of complexion (1), steroid psychosis (1) transient eosinophilia (1)and marked transient oligospermia (1).
No significant change in the laboratory parameters was seen.
| Discussion|| |
With this regimen, it was possible to induce almost every pemphigus patient into a complete remission. Pasricha et al  in their study of 300 patients have also made similar observations. Similar results were reported by Surrinder and Kanwa , in 1990, who treated 50 patients with the same regimen. The same schedule was followed by Appelhans et al  to treat 20 patients with bullous diseases, 11 of whom had pemphigus. Singh et al  in 1996 treated 5 pemphigus vulgaris patients with 136 mg dexamethasone dissolved in 5% glucose. Two patients were also given 500 mg cyclophosphamide on day 1. All the 5 patients treated are in remission.
The duration of phase III in our study was fixed to be 6 months instead of 1 year. None of the patients in this phase had shown any reactivation of the disease. The 12 patients who are at present still in phase I are also expected to go into complete remission in due course of time. All patients in phase I, II and III will continue to receive the required treatment till complete remission is achieved. Patients in phase IV too will be further followed up for any recurrences.
| References|| |
|1.||Pasricha JS, Seetharam KA, Das U. Further studies on pemphigus patients treated with dexamethasone - cyclophosphamide pulse therapy. Ind J Dermatol Venereol Leprol 1989; 55: 98-104. |
|2.||Pasricha JS. Dexamethasone - cyclophosphamide pulse therapy for pemphigus. Int J Dermatol 1995; 34: 875-882. |
|3.||Surrinder K, Kanwar AJ. Dexamethasone - cyclophosphamide pulse therapy in pemphigus. Int J Dermatol 1990; 29: 371-374. |
|4.||Appelhans M, Bonsman G, Orge C, et al. Dexamethasone-cyclophosphamide pulse therapy in bullous autoimmune dermatoses, Hautarzt 1993; 44: 143-147. |
|5.||Singh IP, Mehta SD. Pulse therapy in pemphigus vulgaris. Ind J Dermatol 1996; 41: 31-32. |