ABSTRACT
Background Autoimmune pemphigus is a potentially life threatening bullous disease. The cornerstone of treatment is systemic corticosteroids. However, adjuvant therapy with immunosuppressant drugs is commonly used to improve disease control and alleviate the high morbidity and mortality associated with the use of corticosteroids. Adjunctive treatment with pulse intravenous cyclophosphamide may be more efficacious and less toxic than other immunosuppressants. Objective To retrospectively review the clinical outcome of 18 patients with recalcitrant pemphigus who were treated with cyclophosphamide over the past 10 years. Methodology A retrospective study was conducted between 1985 and 2009 in thirteen Malaysian dermatology centres. Data collected were analysed for comparison of relapse rates, compliance rates and adverse drug effects between the 2 regimes. Results Eighteen patients were included in this audit of which 12 patients had pemphigus vulgaris and 6 patients had pemphigus foliaceous. Prior to treatment with cyclophosphamide, fourteen patients were on azathioprine, three were given intravenous immunoglobulin, and two were prescribed dapsone; however all these patients were either unresponsive, intolerant or suffered serious side-effects with these drugs. Subsequently, 7 patients (median age: 31 years) received a combination of pulse intravenous cyclophosphamide and either intravenous dexamethasone or methylprednisolone. These seven patients received between 2 to 21 pulses of intravenous cyclophosphamide and steroids at monthly intervals with oral prednisolone and cyclophosphamide (50-100mg) in between pulses. The remaining 11 patients (median age: 46 years) received oral cyclophosphamide and corticosteroids. Of the 18 patients in our cohort, 15 achieved control and consolidation of disease activity at an average of 4 weeks and 10 weeks respectively. The remaining three patients are yet to achieve disease control. The total duration of treatment with cyclophosphamide ranged from 2 to 62 months with a cumulative dose ranging from 2.95g to 93.55g. Four patients achieved partial remission on minimal therapy and 3 achieved complete remission. None of patients experienced serious side effects. Conclusion Cyclophosphamide may be an alternative treatment option in patients in patients with pemphigus who fail to respond to standard therapy. Controlled trials are needed to further evaluate the efficacy and safety of this therapy.
ABSTRACT
Pemphigus foliaceous (PF) is an autoimmune blistering disease resulting from acquired immunoglobulin G autoantibodies against desmoglein 1 of the skin, which is one of the adhesion molecules of keratinocytes. Clinically patients with PF develop crusted and scaly erosions mainly over the seborrhoeic distribution i.e. the face, scalp and upper trunk. Mild cases of PF may be localized but in some cases it may progress to erythrodermic exfoliative dermatitis. There is however no mucosal involvement in PF in contrast to pemphigus vulgaris and paraneoplastic pemphigus. Light microscopy of lesional biopsy shows subcorneal acantholysis. Direct immunofluorescence study of perilesional skin reveals presence of intraepithelial intercellular deposit of IgG and C3. We describe 2 cases of PF in the presence of thymoma, a relatively rare association, which could further support the fact of thymoma associated autoimmune disease.