Pulse therapy in pemphigus: report of 11 cases

Abstract

In this study, five cases of pemphigus vulgaris and two cases of pemphigus foliaceus were treated with cyclophosphamide pulse therapy associated with prednisone, resulting in the need for a smaller maintenance dose of prednisone. In three cases of pemphigus vulgaris and one case of pemphigus foliaceus, dexamethasone and cyclophosphamide pulse therapy associated with prednisone helped the lesions to heal more rapidly. Neither treatment however prevented the recurrence of the disease. Amenorrhea, myelotoxicity and Stevens-Johnson syndrome were among the cyclophosphamide side effects. All the patients treated with prednisone experienced known side effects.

Test

“…We have previously observed a homogeneous distribution of PV from the third decade and an increased frequency on the third and sixth decades for PF. The female predominance in pemphigus is in accordance with these series [2,4,5]. In the present investigation, laryngeal lesions and esophageal occurred exclusively in females with PV.…”

“…A peak incidence was found to occur in the fourth decade of life for PV and there was a predominance of the females (Table 1 Prednisone was administered as oral monotherapy (Figure 1) in 7 PV/2 PF cases. In combination with cyclophosphamide pulse therapy: 12 PV (on average ten pulses)/four PF(on average seven pulses); with dexamethasone pulse therapy: ten PV(on average eight pulses)/two PF (on average ive pulses); with hydroxychloroquine: three PV/four PF; with azathioprine: ive PV; with methotrexate: four PV/one PF; with IVIG: two PV ( ive cycles)/two PF (two cycles) [4][5][6]. Patients were divided into two groups according to clinical remission after tenyear follow-up; those treated with prednisone only (n= 9) (Table 2) and those submitted to a combined scheme (n=21) (Table 3).…”

Management of pemphigus vulgaris: challenges and solutions

“… 16 A common pulse regimen used is 100 mg/d of IV dexamethasone for 3 days every 2–3 weeks. 17 However, pulsed CSs do not appear to have additional benefit on top of conventional first-line treatment with oral prednisolone and immunosuppressive adjuvants. 18 Most of the studies investigating the efficacy of pulse steroid treatment involve patients with refractory PV.…”

Oral Manifestations, Gingival Index and Dental State of Vesiculobullous Diseases

“…[17]. The use of cyclophosphamide in pulse therapy associated with prednisone for pemphigus results in a prednisone sparing-effect, as seen in a case of this study [18]. Mycophenolate mofetil in combination with corticosteroids is described as effective in the rapid and complete remission of pemphigus vulgaris [19].…”