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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.
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Background: The diagnosis of pemphigus vulgaris (PV) and pemphigus foliaceous (PF) rests upon clinical, histological and immunofluorescence features. Enzyme-linked immunosorbent assay (ELISA) test and immunoblot (IB) assay have shown variable sensitivity and specificity. Aims: We compared the utility of ELISA and IB in pemphigus patients. Methods: Sixty-six pemphigus cases (PV-54, PF-12) and 72 controls (other vesicobullous disorders and healthy controls) were inducted. ELISA for anti-Dsg 3 and 1 antibodies and IB assay were performed. Results: On ELISA, both mean anti-Dsg 1 and 3 titers were raised in PV and PF. Mean anti-Dsg 1 in mucocutaneous PV was significantly higher than in mucosal PV and mean anti-Dsg 3 was significantly raised in PV than in PF. Anti-Dsg 1 and 3 in the control group were negative. Sensitivity and specificity of ELISA in PV was 98.14% and 90.5% while in PF it was 91.6% and 61.1%, respectively.On IB in PV, 36 cases (66.67%) showed the 130 kDa and 160 kDa antigen bands, 12 (22.2%) only the 130 kDa and six (11.1%) only the 160 kDa band. Eight of the nine pure mucosal cases (88.8%) showed only the 130 kDa. In PF, only the 160 kDa antigen was detected. These antigens were not identified in the control group. Sensitivity and specificity of IB in PV was 88.9% and 100% and in PF it was 100% and 95.2%, respectively. Conclusion: Both tests could differentiate pemphigus from other dermatoses, including other blistering disorders. ELISA could not make a distinction between PV and PF or between the various clinical phenotypes of PV. IB differentiated between PV and PF and the different clinical variants of PV.
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Pênfigo foliáceo é uma doença auto-imune crônica da pele observada com freqüência em cães e, com menor freqüência, em outras espécies animais. Neste trabalho é descrito um caso desta enfermidade no semi-árido da Paraíba, em uma cabra da raça Boer, que apresentava lesões crônicas de pele com extensas áreas alopécicas irregulares, esfoliativas, com crostas amareladas e bilaterais que afetavam a região dorsal da escápula, do tórax, do dorso, da parede do abdominal, da garupa, dos membros pélvicos e da cauda. No rodete coronário, havia espessamento da pele e lesões crostosas. Microscopicamente foram observadas hiperqueratose, espongiose e pústulas intracorneais ou subcornais afetando epiderme e infundíbulos. Nas pústulas, havia grande quantidade de neutrófilos, eosinófilos e células acantolíticas. Na derme, havia acentuada congestão e moderado infiltrado inflamatório composto por eosinófilos, raros plasmócitos, linfócitos e macrófagos, principalmente na periferia de capilares e nos folículos pilosos. Os achados macro e microscópicos são característicos de pênfigo foliáceo. Esta enfermidade é rara em caprinos e, provavelmente, este seja o primeiro relato da doença no Brasil.
Pemphigus foliaceous is an autoimmune disease of the skin affecting mainly dogs and, with less frequency, other domestic species. A case of this disease is reported in the semiarid of Paraiba, Brazil, in a Boer goat with chronic skin lesions with extensive bilateral exfoliatives areas of alopecia, with yellowish crusts affecting the dorsal scapular region, thorax, abdominal wall, croup, hindlimbs and tail. On the coronary band of the hoofs, thick crusts were observed. Microscopiacally, there were hyperkeratosis, spongiosis, intracorneal and sucorneal pustules affecting the epidermis and follicular infundibula. Large number of neutrophils, eosinophils and acantholitic cells were observed in the pustules. The dermis had congestion and mild infiltration of eosinophils, rare plasma cells, lymphocytes and macrophages, mainly in the periphery of follicles. Gross and histologic lesions are characteristic of pemphigus foliaceous, an uncommon disease of goats. This seems to be the first report of pemphigus foliaceous in goats in Brazil.
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No período de agosto de 2000 a julho de 2005 foram atendidos 43 casos de Pênfigo Foliáceo (PF) canino no Serviço de Dermatologia do Hospital Veterinária, Universidade de São Paulo. Com este estudo retrospectivo visou-se atualizar dados referentes à caracterização sexual, definição racial e raça, idade, tipo e topografia lesional, quadro sintomático e resposta aos tratamentos isolados com prednisona e com a associação desta à azatioprina, além de demonstrar o aumento na ocorrência do PF relativamente à série histórica pretérita (1986-2000) do mesmo Serviço.
From August 2000 to July 2005 were attended 43 cases of canine Pemphigus foliaceous (PF) by the Dermatology Service of the Veterinary Teaching Hospital, University of São Paulo. The aim of the present study was to update the records referred to sex, breed, age, type and location of the lesions, clinical signs, and response to treatments with prednisone or combination with prednisone and azathioprine, and also to demonstrate the increase of occurrence of PF compared with the former series (1986-2000) observed in the same Service.