RESUMO
Autoimmune bullous diseases (AIBDs) are a group of rare blistering dermatoses of the mucous membrane and/or skin. The efficacy, safety and treatment durability of intravenous immunoglobulin (IVIg) as an alternative treatment should be explored to systematically review the available literature regarding treatment outcomes with IVIg in AIBD patients. The predefined search strategy was incorporated into the following database, MEDLINE/PubMed, Embase, Scopus and Web of Science on 18 July 2022. Sixty studies were enrolled using Preferred Reporting Items for Systematic Reviews and Meta-analyses guidelines. The use of IVIg alone or combined with rituximab was reported in 500 patients with pemphigus, 82 patients with bullous pemphigoid, 146 patients with mucous membranes pemphigoid and 19 patients with epidermolysis bullosa acquisita. Disease remission with IVIg therapy and RTX + IVIg combination therapy were recorded as 82.8% and 86.7% in pemphigus, 88.0% and 100% in bullous pemphigoid and 91.3% and 75.0% in mucous membrane pemphigoid, respectively. In epidermolysis bullosa acquisita, treatment with IVIg led to 78.6% disease remission; no data were available regarding the treatment with RTX + IVIg in this group of patients. Among all the included patients, 37.5% experienced at least one IVIg-related side effect; the most common ones were headaches, fever/chills and nausea/vomiting. The use of IVIg with or without rituximab had a favourable clinical response in patients with AIBDs. IVIg has no major influence on the normal immune system, which makes its utilization for patients with AIBDs reasonable.
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Doenças Autoimunes , Epidermólise Bolhosa Adquirida , Penfigoide Mucomembranoso Benigno , Penfigoide Bolhoso , Pênfigo , Dermatopatias Vesiculobolhosas , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Epidermólise Bolhosa Adquirida/tratamento farmacológico , Pênfigo/tratamento farmacológico , Rituximab/uso terapêutico , Doenças Autoimunes/tratamento farmacológico , Dermatopatias Vesiculobolhosas/tratamento farmacológicoRESUMO
Over the past few decades, a number of scoring instruments have been developed to assess the severity and activity of autoimmune bullous diseases (AIBDs) (Daneshpazhooh et al., 2019; Nili et al., 2020; Nili et al., 2021; Nili et al., 2022). The Pemphigus Disease Area Index (PDAI), developed by the International Pemphigus Definitions Group, is an easy-to-use, quick, and reliable method for determining pemphigus severity. As a reliable and effective tool in clinical trials, PDAI may also have some limitations and might require some revisions to be used on a daily basis. Here, we propose some recommendations to improve the use of PDAI in the clinical setting.
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OBJECTIVES: This study determines the healing time of lesions on different locations and the contributing factors to the healing time in patients with pemphigus. METHODS: In this prospective study, newly diagnosed patients with mucosal lesions were included. A dermatologist evaluated the lesions, disease status, side effects and assigned the PDAI. Follow-up visits were conducted monthly until the patient reached complete remission and every three months thereafter. A Tzanck smear was performed on lesions clinically suspected to be herpetic in origin. RESULTS: Sixty patients enrolled in the study with a mean age of 45.9 ± 11.7 years. The buccal lesions took the longest to resolve (73[33.5-105.5] days). However, the posterior pharynx lesions showed the shortest healing time (20[13.0-25.5] days). The likelihood of improvement in buccal and soft palate lesions decreased by 5% and 3% with each additional year of age, respectively. Also, the resolution duration of soft palate lesions was significantly shorter in female patients than males (median of 24.0 days vs. 38.5 days). In contrast, lower gingival lesions resolve significantly faster in male patients by a median of 9 days. Herpes simplex virus infection increases the healing time of lesions by 26 days (median of 55 days vs. 29 days, hazard ratio 2.62, 95% CI: 1.04-5.92). CONCLUSIONS: Buccal and lower gingival lesions are more recalcitrant to treatment, while posterior pharynx lesions heal most rapidly. Furthermore, older age was also associated with a lower rate of lesion improvement.
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Herpes Simples , Pênfigo , Humanos , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Pênfigo/patologia , Estudos Prospectivos , Herpes Simples/diagnósticoRESUMO
OBJECTIVES: The existence of standard methods for diagnosis and measuring the severity of diseases leads to a more accurate severity assessment, the possibility of following up, and the possibility of comparing the results of studies. This study aimed to compare different pemphigus vulgaris (PV) assessment methods regarding inter-observer reliability and testing times-focusing on oral parts. MATERIALS AND METHODS: Two dermatologists evaluated orally involved PV patients by oral parts of Autoimmune Bullous Skin Disorder Intensity Score (ABSIS), Pemphigus Disease Area Index (PDAI), and Oral Disease Severity Score (ODSS). RESULTS: Seventy patients completed the study. The intraclass correlation coefficient showed the evaluators' agreements on ABSIS, PDAI, and ODSS with 0.98, 0.94, and 0.95, respectively. Reliability analyses showed near-perfect relationships between each scoring methods pairs. There was no association between lesion sites and disease severity. The PDAI scoring duration was significantly shorter, and the ABSIS scoring duration was significantly longer. CONCLUSION: ODSS is valid for evaluating oral involvement in patients with PV and relates to ABSIS and PDAI almost perfectly. Besides, it was shown that the evaluation of patients' oral involvement based on PDAI and ODSS is done in about 1 min, which seems clinically reasonable.
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Doenças Autoimunes , Doenças da Boca , Pênfigo , Humanos , Pênfigo/diagnóstico , Pênfigo/patologia , Reprodutibilidade dos Testes , Índice de Gravidade de Doença , Doenças da Boca/diagnósticoRESUMO
BACKGROUND: Cutaneous granulomatous disorders (CGDs) can share some features, but an accurate assessment of various findings and their pattern can be useful in differentiating them. In addition to common dermoscopic findings for CGDs, some peculiar dermoscopic characteristics can be helpful in distinguishing them. OBJECTIVE: Herein, we aimed to evaluate dermoscopic findings in patients with CGDs and determine the dermoscopic criteria that could suggest the type of granulomatous disorder. MATERIAL AND METHODS: A total of 107 cases including 75 (70.09%) males and 32 (29.90%) females with an established diagnosis of cutaneous leishmaniasis (n = 49), cutaneous sarcoidosis (n = 23), granuloma annulare (GA) (n = 18), and tattoo granuloma (n = 17) confirmed by clinical and pathological studies were included. Based on the previous studies available in the literature, we wrote a checklist containing dermoscopic features of CGDs. Afterward, two dermatologists independently reviewed all dermoscopic images for the presence or absence of each item on the checklist. Descriptive analysis, fisher exact, chi-square, and t-test were used. The granulomatous disorders with larger sample sizes were selected for further analysis, including the univariate and conditional multivariate logistic regressions. RESULTS: The most prevalent nonvascular findings in all of our CGD patients were white scaling (N = 67%, 62.61%), diffuse or localized orange structureless areas (N = 53%, 49.53%), and diffuse erythema (N = 48%, 44.85%). Furthermore, the most frequent vascular findings in all of our CGD cases were branching and arborizing vessels (N = 30%, 28.03%), linear irregular (N = 30%, 28.03%), and dotted vessels (N = 27%, 25.23%). CONCLUSION: For differentiating leishmaniasis from sarcoidosis by dermoscopy, white scaling and white scarring areas are more suggestive of cutaneous leishmaniasis, whereas the presence of arborizing vessels would be more in favor of sarcoidosis. When comparing GA to cutaneous leishmaniasis, the latter significantly shows more linear irregular vessels, hairpin vessels, white scaling, and white scarring areas. In the case of differentiating sarcoidosis from GA, the presence of hairpin vessels would be suggestive of sarcoidosis.
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Leishmaniose Cutânea , Sarcoidose , Masculino , Feminino , Humanos , Cicatriz/patologia , Dermoscopia , Eritema/patologia , Leishmaniose Cutânea/diagnóstico por imagem , Leishmaniose Cutânea/patologia , Sarcoidose/diagnóstico por imagemRESUMO
BACKGROUND: Seborrheic keratoses (SK) is a benign epithelial skin tumor and plasma exeresis is a new technique. AIMS: To compare the efficacy and safety of plasma exeresis and cryotherapy for treating SK. METHODS: This study is a randomized controlled trial (RCT). One side of each patient was randomly treated with plasma exeresis (peak-to-peak voltage of 3.44 kV and a frequency of 62.5 kHz) and the other side with cryotherapy. RESULTS: Thirty-five males were enrolled. At week 3, 37.1 % (N = 13) of lesions treated by plasma exeresis were clear, which was higher than those treated by cryotherapy 17.1% (N = 6). However, this difference was not significant (p-value: 0.06). At week 6, 16 (57.1 %) out of 28 remaining lesions, treated by plasma exeresis were clear, which was significantly higher (p-value: 0.005) than those completely cleared by cryotherapy in 6 out of 29 remaining lesions (20.7%). The mean physician assessment scale score was significantly reduced in both groups in the second follow-up (plasma group first follow-up 0.91 ± 0.89 vs. second follow-up 0.5 ± 0.64 and p-value: 0.0031; cryo group first follow-up 1.4 ± 0.84 vs. second follow-up 1.1 ± 0.72 and p-value: 0.0002). Regarding side effects, no significant difference was seen (p = 0.438). The most common complications in the plasma and cryotherapy groups were erythema (10/19, 52.63%) and hypo pigmentation (5/13, 38.46%). CONCLUSIONS: Both cryotherapy and plasma exeresis are effective. We observed a significantly higher cleared lesions treated with plasma exeresis in 6 weeks and after two sessions.
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Ceratose Seborreica , Neoplasias Cutâneas , Masculino , Humanos , Ceratose Seborreica/terapia , Crioterapia/efeitos adversos , PigmentaçãoRESUMO
BACKGROUND: Paraneoplastic pemphigus (PNP), also called paraneoplastic autoimmune multiorgan syndrome (PAMS), is a rare autoimmune disease with mucocutaneous and multi-organ involvement. PNP/PAMS is typically associated with lymphoproliferative or haematological malignancies, and less frequently with solid malignancies. The mortality rate of PNP/PAMS is elevated owing to the increased risk of severe infections and disease-associated complications, such as bronchiolitis obliterans. OBJECTIVES: These guidelines summarize evidence-based and expert-based recommendations (S2k level) for the clinical characterization, diagnosis and management of PNP/PAMS. They have been initiated by the Task Force Autoimmune Blistering Diseases of the European Academy of Dermatology and Venereology with the contribution of physicians from all relevant disciplines. The degree of consent among all task force members was included. RESULTS: Chronic severe mucositis and polymorphic skin lesions are clue clinical characteristics of PNP/PAMS. A complete assessment of the patient with suspected PNP/PAMS, requiring histopathological study and immunopathological investigations, including direct and indirect immunofluorescence, ELISA and, where available, immunoblotting/immunoprecipitation, is recommended to achieve a diagnosis of PNP/PAMS. Detection of anti-envoplakin antibodies and/or circulating antibodies binding to the rat bladder epithelium at indirect immunofluorescence is the most specific tool for the diagnosis of PNP/PAMS in a patient with compatible clinical and anamnestic features. Treatment of PNP/PAMS is highly challenging. Systemic steroids up to 1.5 mg/kg/day are recommended as first-line option. Rituximab is also recommended in patients with PNP/PAMS secondary to lymphoproliferative conditions but might also be considered in cases of PNP/PAMS associated with solid tumours. A multidisciplinary approach involving pneumologists, ophthalmologists and onco-haematologists is recommended for optimal management of the patients. CONCLUSIONS: These are the first European guidelines for the diagnosis and management of PNP/PAMS. Diagnostic criteria and therapeutic recommendations will require further validation by prospective studies.
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Síndromes Paraneoplásicas do Sistema Nervoso , Síndromes Paraneoplásicas , Animais , Ratos , Doenças Autoimunes , Neoplasias/complicações , Síndromes Paraneoplásicas/diagnóstico , Síndromes Paraneoplásicas/etiologia , Síndromes Paraneoplásicas/terapia , Síndromes Paraneoplásicas do Sistema Nervoso/diagnóstico , Síndromes Paraneoplásicas do Sistema Nervoso/etiologia , Síndromes Paraneoplásicas do Sistema Nervoso/terapia , Sociedades MédicasRESUMO
Drug-induced cutaneous pseudolymphoma (CPL) is a common form of pseudolymphoma and there are numerous drugs associated with it. In this study, we performed a systematic review of the literature by searching PubMed/Medline and Embase databases to determine the most common drugs responsible for CPL and to define the demographic, clinical, histopathological and immunopathological characteristics of patients (updated on 30 December 2020). From 883 initially found articles, 56 studies (89 reported cases) were included. The mean age of patients was 54.4 ± 17.7 (ranging 8-86) years, and 46 (51.7%) were men. The median time interval between drug intake and CPL occurrence was 120 days (range 1-7300 days). The shortest median time interval between taking the drug and the onset of the disease was observed among patients taking antidepressants (60 days) (range 7-540) and the longest median time interval was observed in individuals using immunomodulators (300 days) (range 3-7300). The most-reported drug categories causing CPL were anti-hypertensives (17.9%), anticonvulsants (14.6%), monoclonal antibodies (13.4%) and antidepressants (11.2%). Moreover, the most common drugs were phenytoin (6.7%), amlodipine (5.6%), fluoxetine (5.6%) and carbamazepine (4.4%). Histopathological evaluation of 76 cases revealed 62 (81.5%) reports of T-cell infiltrations. Furthermore, positive reports of CD4 (94.0%), CD8 (93.0%) and CD30 (87.5%) were noted. The lowest prevalence of CD30-positive reports was observed among monoclonal antibodies. In conclusion, anti-hypertensives, anti-convulsants, monoclonal antibodies and anti-depressants are the most common drugs responsible for CPL. It mostly presents in middle-aged patients with almost no gender difference as pruritic papules, nodules and plaques.
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Pseudolinfoma , Masculino , Pessoa de Meia-Idade , Humanos , Criança , Adolescente , Adulto Jovem , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Pseudolinfoma/induzido quimicamente , Pseudolinfoma/diagnóstico , Anti-Hipertensivos/efeitos adversos , Anticonvulsivantes/efeitos adversos , Carbamazepina/efeitos adversos , Anticorpos Monoclonais/efeitos adversosRESUMO
Pemphigus is a blistering autoimmune disease that is characterized by autoantibodies against desmogleins (Dsg), including anti-Dsg1 and anti-Dsg3. Despite the diagnosis of diseases, the anti-Dsg test by enzyme-linked immunosorbent assay (ELISA) is negative in a small group of pemphigus patients. The aim of this study was to evaluate the clinical course, clinical symptoms, and response to treatment in pemphigus patients with negative levels of anti-Dsg1 and anti-Dsg3. In this study, the data of pemphigus patients referred to Razi Hospital were retrospectively collected from the medical records from 2016 to 2020. Eight patients, whose initial anti-Dsg1/anti-Dsg3 was negative by the ELISA test, were enrolled and their clinical course, clinical signs, and response to treatment were evaluated. The mean age of the subjects (8 females) was 38.75 ± 12.09. The most common phenotype of the subjects was pemphigus vulgaris (PV) with mucosal involvement. Additionally, the common site of blister inception was mouth of the patients. The mean prednisolone dose received by the patients at the initiation was 32.5 ± 13.62 mg/day. According to Pemphigus disease area index (PDAI), six patients had mild severity, while two cases had moderate severity. Among the patients, six subjects received rituximab (RTX). Also, five patients experienced remission after 6.2 ± 5.21 months. PV is the most common phenotype of the disease and mucosal involvement is more common in patients with negative anti-Dsg-1/3 results. The severity of the lesions in most of the patients is mild at baseline and most patients seems to respond to RTX therapy and reach remission.
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Autoanticorpos , Pênfigo , Adulto , Desmogleína 1 , Desmogleína 3 , Feminino , Humanos , Pessoa de Meia-Idade , Pênfigo/diagnóstico , Pênfigo/tratamento farmacológico , Estudos RetrospectivosRESUMO
Pemphigus is a serious autoimmune disease with few appropriate therapeutic options. Although rituximab (RTX) has recently shown great promise in this regard, the best protocol of its administration is remains to be elucidated. This study aimed to evaluate the patients who need at least 3 cycles of treatment with RTX to identify hard-to-treat patients' characteristics, which might lead to consider more prompt protocols for treatment of them. A retrospective cross-sectional study was conducted on 45 patients with pemphigus vulgaris who received at least 3 cycles of RTX. Their demographic, clinical, and laboratory data as well as details of treatment protocol and final clinical situation of patients were evaluated. Totally, 45 patients (21 men and 24 women) with mean age of 44.33 years were included in this paper. Women were about 8 years older than men (mean age: 48.1 years versus 40.1 years, p: 0.011) and needed RTX approximately 2 years later in their course of disease (gap: 41.04 months vs. 14.85 months, p: 0.003). Buccal, truncal, and scalp regions were the most frequent sites of involvement respectively. A significant decrease in both anti-Dsg1, 3 was seen at last visit compared to baseline. However, the amount of this decrement was not significantly different between them (p: 0.083). Partial remission in 31.11%, complete remission in 24.44%, relapse in 15.56%, partial remission on treatment in 15.56% and complete remission on treatment in 13.33% were seen at the last follow-up session. RTX is an effective medication for PV even in patients with refractory disease and its therapeutic effect is increased with each subsequent cycle. Male gender, severe oral mucosal involvement on disease onset and extensive scalp and truncal lesions as first cutaneous manifestation of disease are more likely to be signs of refractory PV. Hence, it is reasonable to consider more prompt protocols for treatment of these cases. Moreover, late prescription of RTX during the course of disease might play a role in presence of more resistant form of disease.
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Pênfigo , Adulto , Estudos Transversais , Feminino , Humanos , Fatores Imunológicos , Masculino , Pessoa de Meia-Idade , Pênfigo/induzido quimicamente , Pênfigo/diagnóstico , Pênfigo/tratamento farmacológico , Estudos Retrospectivos , Rituximab/efeitos adversos , Resultado do TratamentoRESUMO
Rituximab is widely used as the first-line treatment for pemphigus patients. Since it depletes the B cells, it increases the risk of infections. Here, we evaluated the prophylactic efficacy of cotrimoxazole in decreasing the risk of pneumocystis pneumonia (PCP) infection in the pemphigus patients treated with rituximab. The medical records of confirmed pemphigus patients receiving rituximab were evaluated in two groups; those who received cotrimoxazole as a prophylactic after rituximab and patients who only received rituximab without any prophylaxis. The occurrence of PCP infection was determined in each group and compared. Medical records of 494 patients, including 301 women and 193 men, with the mean age of 46.74 years were analyzed. The phenotypes of the disease were mucocutaneous (n = 364), mucosal (n = 88), and cutaneous (n = 42). Among them, 235 cases had received cotrimoxazole as a prophylaxis and 259 patients did not. The incidence of PCP in total patients was 2 (0.4%), one in each group. Accordingly, no significant difference was observed in the incidence of PCP between two groups (p = 0.84). Also, no cotrimoxazole-related side effect was observed in the treated group. It seems that due to the low incidence of PCP in pemphigus patients treated with rituximab, prophylactic cotrimoxazole therapy is not necessary and it only increases the overall therapy cost and might cause cotrimoxazole-related adverse effects in some patients. However, regarding its probable beneficial effect in patients with long-term history of immunosuppressive therapy, more studies are required.
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Pênfigo , Pneumonia por Pneumocystis , Feminino , Humanos , Pênfigo/diagnóstico , Pênfigo/tratamento farmacológico , Pênfigo/prevenção & controle , Pneumonia por Pneumocystis/tratamento farmacológico , Pneumonia por Pneumocystis/etiologia , Pneumonia por Pneumocystis/prevenção & controle , Estudos Retrospectivos , Rituximab , Combinação Trimetoprima e Sulfametoxazol/efeitos adversosRESUMO
Lichen planopilaris (LPP) is a scarring alopecia for which no treatment with remarkable effect has been identified. Pioglitazone has been reported as a possible therapeutic option. To compare the efficacy and safety of pioglitazone with clobetasol in LPP. This randomized, double-blind, parallel-group was conducted at Razi hospital. Patients were treated either with pioglitazone 15 mg/daily or clobetasol lotion 0.05% once at night for 6 months. Patients were visited every 2 months to assess the lichen planopilaris activity index (LPPAI) and record probable adverse events. Forty patients (mean age: 43.6 years; 62.5% female) were randomized 1:1. The mean of LPPAI at baseline and last session were 4.68 ± 1.97 and 2.59 ± 0.97 in the clobetasol group and 5.01 ± 1.71 and 3.04 ± 1.36 in the pioglitazone group, respectively. Both treatments significantly decreased the LPPAI over the two-month interval visits (p < 0.001). No significant difference in the LPPAI reduction was detected between groups. Regarding the safety profile, three clobetasol-treated patients developed folliculitis, and two in the pioglitazone group developed mild headaches. Pioglitazone effectively controlled the signs and symptoms of the LPP with no serious side effects. It can be considered a treatment option for LPP, although it was not superior to clobetasol.
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Clobetasol , Líquen Plano , Humanos , Feminino , Adulto , Masculino , Clobetasol/uso terapêutico , Pioglitazona/efeitos adversos , Resultado do Tratamento , Líquen Plano/diagnóstico , Líquen Plano/tratamento farmacológico , Líquen Plano/induzido quimicamente , Alopecia/tratamento farmacológicoRESUMO
Although the treatments of pemphigus and pemphigoid patients have tended toward safer options, patients with chronic infections seem to be still at the risk of infection reactivation when they are exposed to any of immunosuppressive treatments. A retrospective study on 1646 registered pemphigus and pemphigoid patients was conducted between January 2017 and February 2019 and the prevalence of HBV, the association between the treatments, mainly prednisolone and rituximab with HBV reactivation as well as outcomes of patients after management with antiviral therapies were evaluated. From 1646 reviewed patients, 10 (0.60%) patients with chronic HBV were identified. We found a negative correlation between the ALT (p-value<0.001), AST (p-value = 0.090), and Pemphigus Disease Area Index (PDAI) (p-value = 0.034) and age of patients. At the time points that prednisolone dosage was higher, higher levels of ALT, but no difference in AST levels was noted. The portion of patients with normal ALT was significantly higher (p-value = 0.036; OR = 2.22) in those who had received rituximab within the previous 6 months (38 of 49; 77.6%) as compared to those who did not (81 of 133; 60.9%). We concluded that avoidance (high dose) systemic corticosteroids in patients with chronic HBV, and using rituximab instead in severe cases benefit this group of patients.
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Hepatite B Crônica , Penfigoide Bolhoso , Pênfigo , Humanos , Rituximab/efeitos adversos , Hepatite B Crônica/diagnóstico , Hepatite B Crônica/tratamento farmacológico , Hepatite B Crônica/epidemiologia , Pênfigo/diagnóstico , Pênfigo/tratamento farmacológico , Pênfigo/epidemiologia , Antígenos de Superfície da Hepatite B/uso terapêutico , Penfigoide Bolhoso/diagnóstico , Penfigoide Bolhoso/tratamento farmacológico , Penfigoide Bolhoso/epidemiologia , Prevalência , Estudos Retrospectivos , Ativação Viral , Prednisolona/uso terapêuticoRESUMO
Pemphigus is a group of autoimmune diseases characterized by flaccid lesions on the skin and mucous membranes. In pemphigus vulgaris, the most common subtype of pemphigus, lesions might be appeared anywhere on the oral mucosa, mostly in the buccal mucosa. However, the gingiva is a less frequently affected site. Here, we performed a retrospective study at Tehran University of Medical Sciences, covering a two-year period to identify pemphigus patients with active lesions confined to the gingiva. Considering 1787 initially evaluated pemphigus cases, 512 (28.6%) were found to have a history of gingival involvement. Among them, 31 patients had only gingival involvement during their last visit, including 29 (93.5%) women and only two (6.5%) men. The mean of disease duration in this group was 5.29 ± 3.46 years, and they had gingival involvement for a mean of 23.9 ± 19.3 months. Of 28 patients, nine were negative for anti-Dsg3 and 24 were negative for anti-Dsg1. In 24 patients, who received rituximab, the mean pemphigus disease area index specifically for gingiva was 4.76 ± 0.74 at baseline, which had changed to 4.13 ± 0.75 and 3.26 ± 0.63 three and 6 months after rituximab administration, respectively. After 3 months, gingival lesions were either entirely resolved (n = 3), partially resolved (n = 11), remained unchanged (n = 2), or progressed (n = 7). Gingiva-confined presentation of lesions in pemphigus could be non-anti-Dsg1/3 dependent in some patients. Such patients do not respond well to conventional treatments and rituximab therapy. More studies on the pathogenesis of gingiva-confined presentation of pemphigus are required.
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Pênfigo , Autoanticorpos , Desmogleína 1 , Feminino , Gengiva/patologia , Humanos , Irã (Geográfico) , Masculino , Mucosa Bucal/patologia , Pênfigo/diagnóstico , Pênfigo/tratamento farmacológico , Estudos Retrospectivos , RituximabRESUMO
Rituximab (RTX) combined with short-term glucocorticoids (GC) is an effective therapeutic option for pemphigus. The newly developed Glucocorticoid Toxicity Index (GTI) tool provides the possibility to measure GC toxicities over time. To compare 1-year GTI between two groups of RTX-treated and RTX-naïve patients with pemphigus. The responsiveness of the GTI was also investigated. A prospective cohort of 129 adults with newly diagnosed pemphigus was conducted. GC-related toxicities were assessed at 3-month intervals according to Composite and Specific lists of the GTI. Of the patients, 76.7% (n = 99) received RTX. Throughout the time intervals, RTX-treated patients had lower GTI compared to RTX-naïve ones (p = 0.036). The mean GTI at 1-year was 34.3 in the RTX-treated group and 50.8 in the RTX-naïve group (p = 0.04). The most commonly observed GC-related toxicity was neuropsychiatric manifestations for 34% (224 events). The relapse rate of RTX-treated patients (1%) was significantly lower than RTX-naïve patients (10%) (p = 0.037). The GTI showed no correlation with cumulative GC consumption in both groups (p > 0.05, both). Patients treated with GC alone had remarkably higher GTI than patients treated with GC plus RTX. The GTI is an applicable tool to quantitatively capture GC toxicities at the patient level in pemphigus.
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Pênfigo , Adulto , Humanos , Rituximab/efeitos adversos , Pênfigo/diagnóstico , Pênfigo/tratamento farmacológico , Pênfigo/induzido quimicamente , Glucocorticoides/efeitos adversos , Estudos Prospectivos , Recidiva , Fatores Imunológicos/efeitos adversosRESUMO
The ongoing COVID-19 pandemic has raised concerns regarding the outcome of this infection in patients with autoimmune bullous dermatoses (AIBDs) due to effect of drugs used to treat these disorders. This investigation was performed from the onset of the pandemic to June 1, 2021. Patients with AIBDs who contracted COVID-19 were evaluated. A generalized linear model was employed to find the predictors of severe COVID-19 among patients with AIBDs. Ninety-three patients with AIBDs with a mean age of 50.3 years were evaluated. The most COVID-19 related symptoms were tiredness (76.3%) myalgia (69%), and cough (63.4%). During follow-up, the rate of hospitalization and death were 45.2% and 4.3%, respectively. Previous comorbidities (ß = 0.61) and mean prednisolone dosage above 10 mg/day in the last 3 months (ß = 1.10) significantly increased COVID-19 severity. Also, vaccination against SARS-CoV-2 (ß = -1.50) and each passing month from the last rituximab dose decreased severity (ß = -0.02). Notably, 19.3% of the patients developed AIBD flare-ups following COVID-19 infection. Higher prednisone dose and the shorter interval from the last rituximab infusion were determinants of severe COVID-19. Physicians should assess the risk versus the benefits when prescribing the medications. Moreover, vaccination could successfully attenuate COVID-19 severity.
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Doenças Autoimunes , COVID-19 , Dermatopatias Vesiculobolhosas , Doenças Autoimunes/complicações , Doenças Autoimunes/tratamento farmacológico , Estudos de Coortes , Humanos , Pessoa de Meia-Idade , Pandemias , Rituximab , SARS-CoV-2 , Dermatopatias Vesiculobolhosas/diagnóstico , Dermatopatias Vesiculobolhosas/tratamento farmacológicoRESUMO
BACKGROUND: Corticosteroids have been the mainstay of treatment for alopecia areata (AA). Recently, the 308-nm excimer laser has been proposed for treating AA. OBJECTIVES: To compare the efficacy and safety of excimer laser with intralesional corticosteroid (ILCS) in AA. METHODS: Patients with at least two alopecic patches were randomly assigned to receive weekly excimer laser treatments or monthly injections of ILCS. Photographs and trichoscopy images were examined at baseline, the last treatment session, and after one month of follow-up. The hair regrowth score was evaluated on a 6-point scale. RESULTS: Sixteen patients with 99 alopecic patches completed the study. At the last treatment session, the mean score of hair regrowth for the laser was significantly lower than the ILCS (p = 0.003). However, after a month of follow-up, the difference was not statistically significant (p = 0.148). Positive response in hair regrowth (≥50%) was achieved in 47% of laser-treated patches and 66% in ILCS-treated ones. Four (25%) and 8 (50%) patients experienced severe adverse events of laser and ILCS, respectively. CONCLUSIONS: The excimer laser was safe and effective in AA. The effect of laser on hair regrowth might be delayed as compared with ILCS.
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Alopecia em Áreas , Corticosteroides , Alopecia em Áreas/terapia , Humanos , Lasers de Excimer/uso terapêutico , Resultado do TratamentoRESUMO
AIM: To examine the prevalence of this novel pattern among Iranian patients with pemphigus and peruse the relationship between the presence of a punctate pattern with clinical severity of disease and histopathological findings. METHODS: One hundred recently diagnosed patients with pemphigus were enrolled. DIF evaluation and routine light microscopy were performed on their biopsy specimens. Disease severity was determined using the Pemphigus Disease Area Index. Serum samples were collected to measure autoantibody titers using enzyme-linked immunosorbent assay. RESULTS: All the samples evaluated by DIF showed a continuous linear pattern of intercellular IgG deposition, whereas none of them had a punctate pattern. Despite a significant correlation between the Pemphigus Disease Area Index score and autoantibody values, no association between histopathological findings and disease severity has been found. CONCLUSION: We could not detect any punctate pattern among Iranian patients with pemphigus. The importance of this pattern in the diagnosis of pemphigus might be different among patients with different ethnic and genetic factors.
Assuntos
Autoanticorpos/imunologia , Imunoglobulina G/imunologia , Pênfigo/patologia , Adulto , Desmogleína 1/imunologia , Feminino , Humanos , Irã (Geográfico) , Masculino , Pessoa de Meia-Idade , Pênfigo/diagnóstico , Estudos Prospectivos , Índice de Gravidade de DoençaRESUMO
The COVID-19 has been spreading around the world. Concerns about the safety of administration of immunosuppressive drugs have been raised for treatment of psoriasis (PSO), and there is insufficient evidence for the risk of COVID-19 infection for psoriatic patients using these drugs, so we did a review, focusing on the risk of overall infection associated with the most commonly used immunosuppressive drugs, such as methotrexate, biologics, cyclosporin, Janus kinase inhibitors for the treatment of PSO. The data on the effect of immunosuppressive drugs on this virus may be ever-changing and remains to be clear. We recommend the initiation and continuation of low-risk immunomodulating drugs, such as Interleukin (IL)-17, IL-12/23, and IL-23 inhibitors, for treatment of PSO during COVID-19 era. For psoriatic patients with comorbidities switching to safer modalities such as systemic retinoids, apremilast, and home phototherapy is recommended. Immunosuppressive drugs should be withheld in psoriatic patients with the COVID-19 infection.
Assuntos
COVID-19 , Imunossupressores/uso terapêutico , Psoríase , Humanos , Imunossupressores/efeitos adversos , Pandemias , Psoríase/diagnóstico , Psoríase/tratamento farmacológico , Psoríase/epidemiologia , SARS-CoV-2RESUMO
Pemphigus Vulgaris (PV) is a rare autoimmune blistering disease, which mainly causes mucosal and/or cutaneous lesions. In June 2018, FDA approved Rituximab (RTX)-a B-cell depleting agent-for the management of patients with moderate-to-severe pemphigus. Although the majority of patients respond well to this drug, some do not reach complete remission with a single cycle of RTX. In this review, following an overview of RTX and its clinical outcomes, we have focused on the possible outcomes after RTX therapy in patients with PV. The response is defined into four main categories; complete responders, partial responders, nonresponders, and paradoxical reactions, based on three possibilities of reaching the consolidation phase after 3 months, reaching remission until 6 months, and the ability of corticosteroid tapering in 6 months after RTX administration. Concerning the safety of RTX, three categories of infusion reactions, short and long-term side effects are discussed. Additionally, we have suggested approaches for the evaluation of clinical and serological responses at different critical time-points, including 1, 2, 3, and 6 months after RTX administration. Finally, available markers to predict the response to RTX and research gaps in the field of RTX therapy have been summarized.