RESUMEN
BACKGROUND/OBJECTIVES: Pemphigus vulgaris (PV), as an autoimmune disease including mucosa and the skin, is associated with several complications and comorbidities. The present study planned to determine the effect of L-carnitine (LC) supplementation on biomarkers of oxidative stress (OS), antioxidant capacity and lipid profile in PV patients.Subjects/MethodsFifty two control and patients with PV, participated in the current randomized, double-blind, placebo-controlled clinical trial. The patients were allocated randomly to receive 2 g per day LC tartrate subdivided into two equal doses of 1 g before breakfast and dinner (n=26) or placebo (n=26) for 8 weeks. Anthropometric, lipid profile and OS values were determined at baseline and end of intervention period. RESULTS: LC intake significantly reduced serum levels of triglycerides, total-, LDL- cholesterol and oxidative stress index (OSI; P<0.05). In addition, supplementation with LC resulted to a meaningful increase in levels of total antioxidant capacity (TAC) (P=0.05) and serum carnitine (P<0.001). LC intake revealed non-significant change in serum total oxidant capacity (P=0.15) and HDL- cholesterol (P=0.06) in comparison to the placebo. CONCLUSIONS: LC consumption may have favorable results on TAC, OSI and lipid profiles in patients with PV. The results were in line with the idea that LC supplementation can be associated with positive effects on metabolic status and OS of patients with PV.European Journal of Clinical Nutrition advance online publication, 23 August 2017; doi:10.1038/ejcn.2017.131.
RESUMEN
BACKGROUND: Pemphigus vulgaris (PV) is an autoimmune blistering disorder of the skin and/or mucosa. Increased levels of reactive oxygen species (ROS) were previously reported in PV. AIM: Because oxidative stress has an important role in the inflammatory process, we designed this study to evaluate the antioxidant status in patients with PV and to compare it with that of healthy controls (HCs). METHODS: In this case-control study, 43 newly diagnosed patients with PV were compared with 58 HCs. The severity of the disease was estimated according to Harman scores. Erythrocyte glutathione peroxidase (GPx), superoxide dismutase (SOD), CAT and serum malondialdehyde (MDA) activities and total antioxidant capacity were measured. Data were analyzed by independent t-test. RESULTS: Both groups were similar in sex, age and body mass index. Mean duration of disease was 5.6 months. Mean oral and skin severities were 1.79 and 2.3 respectively, based on Harman scores. SOD activity was not significantly different between groups (1003.30 ± 39.96 vs. 1009.76 ± 32.68 U/gHb). Levels were noticeably higher in patients with PV than in HCs for both GPx (52.13 ± 2.85 vs. 36.63 ± 1.49 U/gHb, respectively; P < 0.001) and CAT (205.69 ± 8.10 vs. 130.26 ± 6.80 kU/gHb, respectively; P < 0.001) activities, and CAT activity correlated with disease severity. In addition, patients had lower total antioxidant capacity than controls (3.39 ± 0.06 vs. 3.72 ± 0.09 mmol/L, P = 0.006). There was no noticeable difference in serum MDA between the two groups (P = 0.45). CONCLUSIONS: Patients with PV have significantly higher antioxidant enzyme activities and lower total antioxidant capacity compared with HCs. These data indicate the importance of improving antioxidant level in patients with pemphigus.
Asunto(s)
Antioxidantes/metabolismo , Pénfigo/enzimología , Adolescente , Adulto , Anciano , Estudios de Casos y Controles , Catalasa/metabolismo , Femenino , Glutatión Peroxidasa/metabolismo , Humanos , Masculino , Malondialdehído/metabolismo , Persona de Mediana Edad , Estrés Oxidativo/fisiología , Pénfigo/metabolismo , Superóxido Dismutasa/metabolismo , Adulto JovenRESUMEN
BACKGROUND: Pemphigus vulgaris (PV) is a known cause of loss of 'normal' anagen hair; that is, shedding of intact anagen hairs covered by root sheaths. However, studies on this subject are limited. AIM: To investigate anagen hair shedding in patients with PV, and ascertain its association with disease severity. METHODS: In total, 96 consecutive patients with PV (new patients or patients in relapse) who were admitted to the dermatology wards of a tertiary hospital were enrolled in this study. Demographic data, PV phenotype, disease severity and presence of scalp lesions were recorded. A group of 10-20 hairs were pulled gently from different areas of the scalp (lesional and nonlesional skin) in all patients, and anagen hairs were counted. Disease severity was graded according to Harman score. RESULTS: Anagen hair was obtained by pull test in 59 of the 96 patients (61.5%), of whom 2 had normal scalp. The mean ± SD anagen hair count was 5.9 ± 7.6 (range 0-31). In univariate analysis, anagen hair loss (P < 0.01) and the presence of scalp lesions (P = 0.01) were associated with severe disease. Mean anagen hair count was significantly higher in the severe (mean 6.83 ± 7.89) than the moderate (mean 1.06 ± 1.94) subgroup (P < 0.001). Multivariate analysis confirmed anagen hair loss (OR = 1.16, 95% CI = 1.05-1.28, P < 0.01), but not scalp lesions (P = 0.69) as an independent predictor of disease severity. CONCLUSIONS: According to our study, normal anagen effluvium is a frequent finding in patients with PV, and interestingly, this was observed in nonlesional as well as lesional scalp. In addition, severe anagen hair loss was an independent predictor of the disease severity.
Asunto(s)
Alopecia/etiología , Pénfigo/complicaciones , Dermatosis del Cuero Cabelludo/complicaciones , Adulto , Anciano , Alopecia/patología , Femenino , Cabello/crecimiento & desarrollo , Humanos , Masculino , Persona de Mediana Edad , Análisis Multivariante , Pénfigo/patología , Fenotipo , Dermatosis del Cuero Cabelludo/patologíaRESUMEN
BACKGROUND: Bullous pemphigoid (BP) is a subepidermal blistering disease, characterized by autoantibodies directed against BP180 and BP230. Collecting saliva is an easy and painless way of obtaining biological samples, and can be used for diagnosis of autoimmune diseases. AIM: To compare the diagnostic accuracy of serum and salivary BP180-NC16a and BP230-C3 in the initial diagnosis of BP. METHODS: We assessed 50 patients newly diagnosed with BP and 50 healthy controls. The diagnosis of BP was confirmed based on clinical, histopathological and immunofluorescence findings. Serum and saliva samples were collected from both groups, and BP180 and BP230 titres were assessed using commercially available ELISA kits. RESULTS: Using serum, the sensitivity of the serum BP180 and BP230 ELISA assays was 88% and 48%, respectively, and the specificity of both was 96%. Using saliva with the cutoff value proposed by the manufacturer, sensitivity was 56.2% and 14.6%, and specificity was 98% and 100%, respectively. Using the best calculated cutoff for saliva, sensitivity increased to 87.5% and 77.1%, and specificity to 96% and 62%, respectively. There was a significant correlation between serum and saliva BP180 levels and the severity of skin disease. Both serum and saliva BP230 levels were significantly higher in patients with mucosal involvement. CONCLUSION: Serum BP180 NC16a ELISA is a sensitive and specific test for the initial diagnosis of BP, whereas serum BP230-C3 ELISA is highly specific, but less sensitive. Saliva may be a noninvasive and convenient alternative for use in the BP180 NC16a ELISA to diagnose BP.
Asunto(s)
Autoantígenos/análisis , Proteínas Portadoras/análisis , Proteínas del Citoesqueleto/análisis , Proteínas del Tejido Nervioso/análisis , Colágenos no Fibrilares/análisis , Penfigoide Ampolloso/diagnóstico , Adulto , Anciano , Anciano de 80 o más Años , Autoantígenos/sangre , Biomarcadores/análisis , Biomarcadores/sangre , Proteínas Portadoras/sangre , Estudios de Casos y Controles , Proteínas del Citoesqueleto/sangre , Distonina , Ensayo de Inmunoadsorción Enzimática , Femenino , Humanos , Masculino , Persona de Mediana Edad , Proteínas del Tejido Nervioso/sangre , Colágenos no Fibrilares/sangre , Penfigoide Ampolloso/inmunología , Análisis de Regresión , Saliva/química , Sensibilidad y Especificidad , Colágeno Tipo XVIIRESUMEN
BACKGROUND: Drug withdrawal is the ultimate goal in the management of patients with pemphigus. Direct immunofluorescence (DIF) has long been considered the gold-standard test to predict immunological remission in pemphigus vulgaris (PV); however, there have been no comparisons between DIF and antidesmoglein (anti-Dsg) ELISA. AIM: To compare anti-Dsg ELISA with DIF in patients with PV for evaluation of immunological remission. METHODS: The study enrolled 46 patients with PV who had absence of any lesion, and had a daily prednisolone dosage of ≤ 10 mg without adjuvant drug treatment in the preceding 6 months. Biopsy specimens were taken from patients and processed for DIF. Intercellular deposition of IgG and/or C3 was considered positive. Serum samples were also collected for anti-Dsg1 and anti-Dsg3 ELISA, and an ELISA index value of > 20.0 was considered positive. RESULTS: DIF and anti-Dsg ELISA were positive for 11 (23.9%) and 18 patients (39.1%), respectively. Anti-Dsg ELISA had a sensitivity of 100%, a specificity of 80%, a positive predictive value of 61.1% and a negative predictive value of 100%. CONCLUSIONS: The high sensitivity of anti-Dsg ELISA proves that this simple serological test is a good substitute for DIF for evaluation of immunological remission in PV. As none of the DIF-positive patients was anti-Dsg-negative, it is possible that during the course of immunological remission, results for DIF may become negative before the results for Dsg ELISA do so.
Asunto(s)
Autoanticuerpos/sangre , Desmogleína 1/inmunología , Desmogleína 3/inmunología , Ensayo de Inmunoadsorción Enzimática/métodos , Técnica del Anticuerpo Fluorescente Directa , Pénfigo/inmunología , Adolescente , Adulto , Anciano , Autoanticuerpos/inmunología , Femenino , Glucocorticoides/uso terapéutico , Humanos , Inmunoglobulina G/inmunología , Masculino , Persona de Mediana Edad , Pénfigo/tratamiento farmacológico , Valor Predictivo de las Pruebas , Prednisolona/uso terapéutico , Sensibilidad y Especificidad , Adulto JovenRESUMEN
Systemic corticosteroids have long been the mainstay of treatment for pemphigus patients. However, the necessity of their high-dose and long-term administration has brought about a number of complications, sometimes causing significant morbidities. Maintaining a balance between therapeutic and undesirable effects of medications is not always easily achievable. Therefore, additional treatment modalities are frequently needed to control side-effects. Kaposi's sarcoma (KS) is a rare, potentially life-threatening complication in this setting. Due to the rarity of data on pemphigus-associated KS treatment, the best therapeutic approach is still undecided. Here, we report two cases of pemphigus patients who had developed extensive KS as a result of severe immunosuppressive therapy and were successfully treated with paclitaxel. In addition, we performed a review of literature to assess the results of the previously employed treatment modalities in this setting.
Asunto(s)
Paclitaxel/uso terapéutico , Pénfigo/complicaciones , Sarcoma de Kaposi/complicaciones , Humanos , Inmunosupresores/uso terapéutico , Masculino , Persona de Mediana Edad , Pénfigo/tratamiento farmacológicoRESUMEN
BACKGROUND: The classic treatment for pemphigus vulgaris is prednisolone. Immunosuppressive drugs can be used in association. OBJECTIVE: To compare the efficacy of Azathioprine in reducing the Disease Activity Index (DAI). PATIENTS AND METHODS: A double blind randomized controlled study was conducted on 56 new patients, assigned to two therapeutic groups: (i) prednisolone plus placebo; (ii) prednisolone plus Azathioprine. Patients were checked regularly for 1 year. 'Complete remission' was defined as healing of all lesions after 12 months, and prednisolone <7.5 mg daily, (DAI ≤ 1). Analysis was done by 'Intention To Treat' (ITT) and 'Treatment Completed Analysis' (TCA). RESULTS: Both groups were similar in age, gender, disease duration, and DAI. Primary endpoint: By ITT and TCA, the mean DAI improved in both groups with no significant difference between them. The difference became significant for the last trimester (3 months; ITT: P = 0.033, TCA: P = 0.045). Secondary endpoint: The total steroid dose decreased significantly in both groups, with no significant difference between them, except for the last trimester (ITT: P = 0.011, TCA: P = 0.035). The mean daily steroid dose decreased gradually in both groups becoming statistically significant in favour of azathioprine, in the last trimester, especially at 12th months (ITT: P = 0.002, TCA: P = 0.005). Complete remission was significant at 12 months only for TCA (AZA/Control: 53.6%/39.9%, P = 0.043). LIMITATIONS: Sample size was rather small to demonstrate all differences. Other limitations include the choice of primary and secondary endpoints and the unavailability to measure thiopurine methyltransferase activity. CONCLUSION: Azathioprine helps to reduce prednisolone dose in long-run.
Asunto(s)
Azatioprina/uso terapéutico , Pénfigo/tratamiento farmacológico , Prednisolona/uso terapéutico , Índice de Severidad de la Enfermedad , Adulto , Relación Dosis-Respuesta a Droga , Método Doble Ciego , Quimioterapia Combinada , Determinación de Punto Final , Femenino , Estudios de Seguimiento , Humanos , Masculino , Persona de Mediana Edad , Resultado del TratamientoRESUMEN
BACKGROUND: Pemphigus vulgaris is a life threatening, blistering skin disease. It is an autoimmune abnormality. Due to involvement of oral cavity and pharynx, patients are at risk of nutrients deficiency. The aim of this study was to evaluate the status of selenium, copper, and zinc in these patients. METHODS: In a case-control study, 43 newly diagnosed pemphigus vulgaris patients were compared with 58 healthy people from 2009 to 2010. The severity of the disease was estimated according to Harman's scores. Serum selenium was measured with atomic absorption but serum zinc and copper concentrations were determined spectrophotometrically. Data were compared with independent t test. Correlations were evaluated by Pearson correlation test. RESULTS: Both groups were the same based on sex, age, and weight and body mass index. The mean duration of disease was 5.6 month. The oral and skin severities were 1.79 and 2.3 respectively, based on Harman's scores. Serum selenium of pemphigus patients was significantly less than that of healthy people (P<0.001). Serum copper was negatively correlated with duration of disease in males (P=0.02, r=-0.5). CONCLUSIONS: Pemphigus vulgaris negatively affects on serum selenium, copper and zinc. It seems that serum selenium, copper and zinc decrease as the disease lasts longer.
RESUMEN
BACKGROUND: Methotrexate (MTX) is a well-known systemic drug for moderate to severe chronic plaque psoriasis. Recently, mycophenolate mofetil (MMF) has been recommended for psoriasis. OBJECTIVE: To compare the efficacy and safety of MMF vs. MTX for the treatment of chronic plaque psoriasis. METHODS: Thirty-eight consecutive patients with Psoriasis Area and Severity Index (PASI)>10 were randomly assigned for 12 weeks of treatment with either MTX (18 patients; initial dose, 7.5 mg/week) or MMF (20 patients; dose; 2 g/day) and were followed for 12 weeks after discontinuing the treatment. The differences between the two groups were analysed at the end of treatment and follow-up comparing with baseline values. RESULTS: After 12 weeks of treatment, the mean ± SD score for the PASI decreased from 16.46 ± 5.29 at baseline to 3.17 ± 2.35 among 15 patients treated with MTX, whereas the score decreased from 17.43 ± 7.42 to 3.97 ± 5.95 among 17 patients treated with MMF (P>0.05). Twelve weeks after discontinuing the treatment, the scores were 4.77 ± 3.52 and 5.94 ± 4.27, respectively (P>0.05). PASI -75 were achieved in 58.8% of patients in MMF group and 73.3% in MTX group (P > 0.05). Three months after discontinuing the treatment, PASI-75 remained in 33.3% of patients in MMF and 53.3% of MTX group (P > 0.05). Both drugs were well tolerated and side-effects were minor and transient. CONCLUSIONS: No significant differences in efficacy were found between MTX and MMF groups. MMF may represent a good alternative for the treatment of psoriasis in patients who are unable to take MTX or other available drugs due to contraindication or toxicity.
Asunto(s)
Fármacos Dermatológicos/uso terapéutico , Metotrexato/uso terapéutico , Ácido Micofenólico/análogos & derivados , Psoriasis/tratamiento farmacológico , Adolescente , Adulto , Enfermedad Crónica , Fármacos Dermatológicos/efectos adversos , Femenino , Humanos , Masculino , Metotrexato/efectos adversos , Persona de Mediana Edad , Ácido Micofenólico/efectos adversos , Ácido Micofenólico/uso terapéutico , Resultado del Tratamiento , Adulto JovenRESUMEN
BACKGROUND: To the best of our knowledge there is only one report about salivary desmoglein (Dsg) 1 and 3 enzyme-linked immunosorbent assay (ELISA) in pemphigus vulgaris (PV), whereas several studies have been performed on serum. AIMS: To find the sensitivity of serum and salivary anti-Dsg1 and 3 antibodies in the diagnosis of PV, and to determine the relationship between disease severity and phenotype with antibody levels. METHODS: Fifty new patients with PV were included in this study. The diagnosis of PV was confirmed by histopathology and direct immunofluorescence. Demographical data, disease severity and phenotypes were recorded on questionnaire sheets. Dsg1 and Dsg3 ELISA were performed on serum and salivary samples of patients and controls. RESULTS: Thirty-seven patients had mucocutaneous phenotype; whereas mucosal dominant and cutaneous dominant phenotypes were seen in 11 and 2 patients respectively. The sensitivities of serum anti-Dsg3 and anti-Dsg1 were 94% and 72% respectively. The sensitivities of salivary anti-Dsg3 and anti-Dsg1 antibodies were accordingly 94% and 70%. Compared with mucosal phenotype, serum and salivary anti-Dsg1 antibodies were significantly higher in the patients with mucocutaneous phenotype. Serum Dsg1 antibodies were related with cutaneous and serum Dsg3 antibodies with mucosal severity scores. Salivary Dsg1 antibodies were significantly correlated with mucosal severity (P=0.00); however there was no correlation between this antibody and cutaneous severity (P=0.07). Salivary Dsg3 antibodies were not correlated with mucosal severity (P=0.16). CONCLUSION: Saliva Dsg ELISA could be used for diagnosis of PV. Salivary Dsg1 antibodies had a significant correlation with mucosal severity.
Asunto(s)
Desmogleína 1/metabolismo , Desmogleína 3/metabolismo , Ensayo de Inmunoadsorción Enzimática , Pénfigo/metabolismo , Saliva/metabolismo , Adolescente , Adulto , Anciano , Autoanticuerpos/inmunología , Autoanticuerpos/metabolismo , Biomarcadores/metabolismo , Desmogleína 1/inmunología , Desmogleína 3/inmunología , Femenino , Humanos , Masculino , Persona de Mediana Edad , Pénfigo/diagnóstico , Pénfigo/inmunología , Fenotipo , Estudios Retrospectivos , Índice de Severidad de la Enfermedad , Adulto JovenRESUMEN
BACKGROUND: Pemphigus vulgaris (PV) usually presents as non-healing, painful oral erosions, but transient or aphtha-like lesions are not exceptional at the very beginning of the disease leading to the common misdiagnosis of recurrent aphthous stomatitis (RAS). We designed this cross-sectional, questionnaire-based study to evaluate this underreported yet important presentation of PV. MATERIAL AND METHODS: One hundred and eighty-five consecutive PV patients were interviewed and a questionnaire, comprising items related to the natural history of oral lesions, was filled in for each. Fourteen patients who had taken steroids (topical or systemic) before their final diagnosis were excluded. RESULTS: Twenty-three per cent of patients gave a history of transient aphthous-like lesions; 95% of them were misdiagnosed as aphthae. These lesions were especially reported by patients aged 40 years or older (P < 0.047). CONCLUSION: PV should be kept in mind as a rare differential diagnosis of transient oral ulcerations. We recommend careful observation of these patients and performing indirect immunofluorescence or desmoglein ELISA and even biopsy in atypical cases, to rule out PV especially in older patients and predisposed ethic groups. To find out the differential aspects of RAS and aphthous-like PV, a cohort study on RAS patients is suggested.
Asunto(s)
Pénfigo/diagnóstico , Estomatitis Aftosa/diagnóstico , Adulto , Estudios Transversales , Diagnóstico Diferencial , Ensayo de Inmunoadsorción Enzimática , Femenino , Técnica del Anticuerpo Fluorescente Indirecta , Humanos , Masculino , Persona de Mediana Edad , Pénfigo/fisiopatología , Estomatitis Aftosa/fisiopatología , Encuestas y CuestionariosRESUMEN
BACKGROUND: It has recently been demonstrated in a study on 15 patients that plucked hair can be used as a substrate for direct immunofluorescence (DIF) in pemphigus. OBJECTIVE: Our aim was to assess the sensitivity of DIF on plucked hairs in pemphigus vulgaris (PV) patients with positive DIF of oral mucosa. METHODS: One hundred and ten new PV patients were enrolled in the study. They all showed the typical clinical and histological findings as well as positive DIF of the oral mucosa, diagnostic for PV. Approximately 30 hairs were obtained in the same way as for the trichogram. The hairs with their outer root sheaths (ORS) were processed for DIF in order to detect immunoglobulin G and C3. RESULTS: Immunodeposits favouring PV were demonstrated in the ORS of 100 cases showing a sensitivity of 91%. CONCLUSION: Regarding the relatively high sensitivity of DIF on plucked hair in PV patients with positive oral mucosal DIF in our study, it seems that hair plucking is a suitable alternative to the more invasive techniques of skin or mucosal biopsy for obtaining specimens for DIF in PV.
Asunto(s)
Cabello , Pénfigo/etiología , Femenino , Técnica del Anticuerpo Fluorescente Directa , Humanos , Masculino , Mucosa Bucal/patología , Sensibilidad y EspecificidadRESUMEN
BACKGROUND: Pemphigus vulgaris can be divided into mucosal, mucocutaneous and cutaneous subtypes. A higher mortality rate has been shown with mucocutaneous involvement. OBJECTIVES: The aim of this retrospective study was to analyse the association of clinical subtypes of pemphigus vulgaris with remission rates. PATIENTS/METHODS: One hundred twenty-eight patients with pemphigus vulgaris, treated with prednisolone 2 mg/kg/day plus azathioprine 2 to 2.5 mg/kg/day, were enrolled. The partial and complete remission rates, at the end of the first and second years of treatment, and the number of relapses were compared in the three groups. RESULTS: After disease establishment, 71.1% had mucocutaneous, 18.8% had mucosal, and 10.2% had only cutaneous involvement. The mean duration of follow-up was 53.5 +/- 39.6 months. The mean duration it took the mucocutaneous group to reach a prednisolone dosage of 30 mg/day was significantly longer (P = 0.050). Mucocutaneous patients had a significantly lower rate of remission (31.9%) compared with those with only mucosal or cutaneous involvement (48.6%) at the end of the first year of the treatment (P = 0.029). After 2 years, mucocutaneous patients again had a lower remission rate (32.9% vs. 44.5%). Relapses were also more frequent in this subtype. Those presenting with mucosal or mucocutaneous erosions had a higher rate of active disease after receiving treatment for a year compared with those with only cutaneous presentation (66.7% vs. 45%; P = 0.057). CONCLUSIONS: In mucocutaneous subtype, clinical control was achieved later, and they had a lower rate of remission at the end of the first and second years of treatment. They were also prone to relapses.
Asunto(s)
Azatioprina/uso terapéutico , Glucocorticoides/uso terapéutico , Inmunosupresores/uso terapéutico , Pénfigo/clasificación , Pénfigo/tratamiento farmacológico , Prednisolona/uso terapéutico , Adulto , Progresión de la Enfermedad , Relación Dosis-Respuesta a Droga , Quimioterapia Combinada , Femenino , Humanos , Masculino , Persona de Mediana Edad , Pénfigo/patología , Estudios Retrospectivos , Prevención Secundaria , Factores de Tiempo , Resultado del TratamientoAsunto(s)
Pénfigo/patología , Progresión de la Enfermedad , Femenino , Humanos , Masculino , Fenotipo , Factores de TiempoRESUMEN
BACKGROUND: Pemphigus vulgaris (PV) is an autoimmune blistering disease which is known to involve the female genital tract, but the frequency at which this occurs is unknown. There are few reports in the literature of the cytological appearance of PV on cervicovaginal smears. OBJECTIVES: To evaluate involvement of the female genital tract and the appearance of cervicovaginal Papanicolaou (Pap) smears in PV. METHODS: The study included 77 patients with PV who attended between April 2005 and February 2007. Each patient was subjected to gynaecological examination and cervicovaginal Pap smear. RESULTS: Genital lesions were observed in 39 patients (51%). Of these, the labia minora were involved in 36 patients (92%), the labia majora in 11 (28%), the vagina in 14 (36%) and the cervix in six (15%). Cervicovaginal Pap smears of 20 of 77 patients (26%) showed PV. Of 72 satisfactory Pap smears, the cervical Pap smear was normal in 25 patients (35%), inflammatory in 43 patients (60%), and dysplastic (low-grade squamous intraepithelial neoplasia) in four patients (6%). CONCLUSIONS: Involvement of the female genital tract with PV might not be as infrequent as was previously thought; it is probably the second most common mucosal site of PV after the oral mucosa. Genital lesions may be missed and the need for thorough pelvic examination should not be overlooked.
Asunto(s)
Cuello del Útero/patología , Pénfigo/patología , Adulto , Anciano , Anciano de 80 o más Años , Femenino , Humanos , Persona de Mediana Edad , Prueba de Papanicolaou , Lesiones Precancerosas/patología , Prevalencia , Resultado del Tratamiento , Frotis Vaginal/métodosRESUMEN
BACKGROUND: Pemphigus vulgaris (PV) is a chronic autoimmune blistering disorder of the skin and mucosa characterized by the presence of autoantibodies against desmoglein3 (Dsg3). Some patients also have antibodies against desmoglein1 (Dsg1). The aims of this study were to evaluate the diagnostic value of Dsg enzyme-linked immunosorbent assay (ELISA) in Iranian PV patients, to assess its correlation with the clinical phenotype and severity of disease and to investigate the changes of these antibodies after treatment. METHODS: Seventy-three patients with PV (29 men, 44 women) presenting to the Pemphigus Research Unit at Razi Hospital, Tehran, Iran were enrolled. ELISAs were used to detect IgG autoantibodies reactive with the ectodomains of Dsg1 and Dsg3, and the correlation of antibodies with the clinical phenotype as well as oral and skin disease severity was assessed. In addition, the tests were repeated in 18 patients after treatment and the resulting remission. RESULTS: Anti-Dsg1 and anti-Dsg3 were detected in 56 (76.7%) and 69 (94.5%) patients, respectively. Anti-Dsg1 and anti-Dsg3 antibodies were present in 48 (94.1%) and 50 (98%) patients with mucocutaneous type, in 2 (12.5%) and 15 (93.7%) patients with mucosal type, and in 6 (100%) and 4 (66.7%) patients with cutaneous PV, respectively. The mean anti-Dsg1 index values were significantly higher in cutaneous and mucocutaneous phenotypes than mucosal PV (P < 0.001). The mean anti-Dsg3 index values were significantly lower in cutaneous and mucosal phenotypes than mucocutaneous PV (P < 0.01). The severity of skin lesions (but not oral lesions) was correlated with anti-Dsg1 antibody level (P < 0.001); on the other hand, the severity of oral lesions (P < 0.01) as well as skin lesions (P < 0.001) was significantly correlated with anti-Dsg3 antibody levels. Both anti-Dsg1 and anti-Dsg3 levels were significantly reduced after treatment and clinical remission (P < 0.001). CONCLUSION: Dsg ELISA is not only a sensitive tool for the diagnosis of PV, it can also serve as a predictive means for assessing the severity as well as for monitoring the disease activity. Although, in general, the clinical phenotype is related to the antibody profile, there are occasional cases with discordant phenotype and antibody profile. These discrepancies might be explained by genetic variations or the presence of possible minor antigens involved in the pathogenesis of pemphigus.
Asunto(s)
Desmogleína 1/metabolismo , Desmogleína 3/metabolismo , Ensayo de Inmunoadsorción Enzimática/métodos , Pénfigo/diagnóstico , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Análisis de Varianza , Biomarcadores/metabolismo , Niño , Desmogleína 1/inmunología , Desmogleína 3/inmunología , Femenino , Humanos , Irán , Masculino , Persona de Mediana Edad , Pénfigo/inmunología , Pénfigo/patología , Fenotipo , Valor Predictivo de las Pruebas , Sensibilidad y Especificidad , Índice de Severidad de la Enfermedad , Estadísticas no ParamétricasRESUMEN
BACKGROUND: Recent reports have revealed the relatively high incidence of pemphigus in Iran. Occupational exposure and personal habits have been suggested to play a role in the aetiopathogenesis of this life-threatening disease. AIM: In order to analyse the association of environmental factors with pemphigus, we conducted a case-control study to evaluate the possible role of smoking, pesticide exposure and hormonal factors in Iran. METHODS: This study was conducted in Iran using a structured questionnaire. Questions included information on patients' smoking habits, occupational exposure to pesticides, use of oral contraception (OC) and number of pregnancies. RESULTS: We enrolled 210 patients with pemphigus and 205 control subjects. Fewer of patients with pemphigus (17.1%) reported a current or past history of smoking, which was statistically different from the control group (27.3% smokers). The duration of smoking and the number of cigarettes smoked daily was also significantly lower in patients. Although OC use was significantly higher in women with pemphigus, the mean number of pregnancies was not different between the two groups. Occupational exposure to pesticides was significantly higher in patients with pemphigus (14.8%) than in controls (5.4%); patients with pemphigus were exposed to pesticides three times more often than were healthy subjects. CONCLUSION: As a positive history of smoking was lower in patients with pemphigus compared with healthy subjects, it seems that smoking is a protective factor in pemphigus. This should encourage further investigations, searching for novel therapies. If pesticides and OC are confirmed as triggering factors, their cessation might reduce the need for pharmacological therapy.
Asunto(s)
Anticonceptivos Hormonales Orales/efectos adversos , Exposición Profesional/efectos adversos , Pénfigo/inducido químicamente , Plaguicidas/toxicidad , Fumar/efectos adversos , Adulto , Estudios de Casos y Controles , Femenino , Humanos , Irán/epidemiología , Masculino , Persona de Mediana Edad , Pénfigo/epidemiología , Embarazo , Factores de Riesgo , Encuestas y CuestionariosRESUMEN
BACKGROUND: Pemphigus vulgaris (PV) is a severe blistering disease involving the skin and mucous membranes. The most common causes of death in these patients are adverse effects of drugs, and infection. Skin lesions are one of the important sources of infection. Thus, any local treatment that could reduce healing time of lesions and consequently reduce the total dosage of drugs needed to treat is favourable. OBJECTIVE: To evaluate the efficacy of epidermal growth factor (EGF) in reducing healing time of lesions in patients with pemphigus vulgaris. METHODS: In this randomized, double-blind, within-patient, left/right, controlled trial, 20 hospitalized patients with pathologial and immunohistologial (direct and indirect immunoflourecence) proven pemphigus vulgaris (PV) were chosen. In addition, all patients had at least one appropriate pemphigus lesion on each side of the body that had not healed after 2-week systemic therapy and sterile saline washing. EGF (10 microg/g) in 0.1% silver sulfadiazine cream vs. 0.1% silver sulfadiazine cream alone was applied randomly on one side of the body. RESULTS: Kaplan-Meier survival analysis suggested that median time to heal with application of EGF plus silver sulfadiazine cream was 9 days, in comparison with 15 days for silver sulfadiazine cream alone (log-rank test, P=0.0003). No intervention-related adverse effect was observed during the study. CONCLUSIONS: EGF can significantly reduce healing time of skin lesions in patients with pemphigus vulgaris, at least when this cream base is applied (Cochrane skin group identifier: CSG20).
Asunto(s)
Factor de Crecimiento Epidérmico/uso terapéutico , Pénfigo/tratamiento farmacológico , Adulto , Anciano , Método Doble Ciego , Factor de Crecimiento Epidérmico/farmacología , Femenino , Humanos , Masculino , Persona de Mediana Edad , Cicatrización de Heridas/efectos de los fármacosRESUMEN
Although early stage mycosis fungoides (MF) has a generally good prognosis, and long-term survival rates with current therapies (UVB, photochemotherapy, topical nitrogen mustard, electron beam radiotherapy) are similar, there is concern regarding their potential side effects. It has been reported that the same effective UVB dose is safer than PUVA in terms of carcinogenicity, and that it produces fewer side effects. Our aim was to evaluate the effect of narrow-band UVB in the treatment of early stage MF. Sixteen patients (seven males, nine females; mean age, 40 years) with early stage MF received TL-01 phototherapy three times per week using a standard protocol. Twelve patients (75%) had complete response in a mean of 27.9 treatments, three had partial response, and one no response. Upon discontinuation of treatment, six patients with complete response relapsed in a mean time to relapse of 4.5 months. The present study indicates that narrow-band-UVB is an effective treatment modality for early stage MF.