B淋巴细胞激活因子及其受体mRNA与系统性红斑狼疮关联性研究

目的:研究建立实时荧光定量(RFQ)-PCR法测定B淋巴细胞激活因子(BLyS)及其受体mRNA含量的方法,探讨其在系统性红斑狼疮(SLE)患者外周血单一核细胞(PBMC)中表达的检测价值。方法:在BLyS及其受体基因保守区设计特异性的引物和荧光探针,用反转录(RT)-PCR扩增目的片段实时检测产物的荧光强度,用所建立的RFQ-PCR方法检测23例SLE患者、23名正常人外周血BLyS及其受体mRNA表达。结果:SLE患者B淋巴细胞成熟抗原(BCMA)、穿膜蛋白活化物(TACI)和B淋巴细胞活化因子受体(BAFF)-RmRNA的表达明显高于正常人(P<0.01),活动期患者的表达高于缓解期(P<0.01),抗ds-DNA抗体阳性和尿蛋白阳性的SLE患者BCMA、TACI和BAFF-RmRNA的表达高于阴性患者(P<0.01)。结论:RFQ-PCR检测BLyS及其受体mRNA含量的方法具有较好的灵敏度和重复性;SLE患者BLyS、TACI和BAFF-RmRNA表达含量升高,提示BLyS、TACI和BAFF-R可能与SLE的发病有关。     

[Translated article] Oral Lesions in Patients With Psoriasis: Prevalence and Association With Its Clinical and Epidemiological Characteristics

Background and objectivePsoriasis is a multisystem disease associated with an increased prevalence of oral lesions. The aim of this study was to determine the prevalence of oral lesions in patients with psoriasis and examine associations with clinical and patient characteristics.Material and methodsWe conducted a cross-sectional study of patients with psoriasis and healthy controls seen between December 2019 and February 2020. We recorded biometric data, comorbidities associated with psoriasis, oral examination findings, and clinical characteristics of psoriasis.ResultsWe studied 100 patients with psoriasis and 100 controls. Oral lesions were more common in the psoriasis group (74% vs 46%, P < .001). The most common lesions were fissured tongue (39% vs 16%, P < .001) and periodontitis (28% vs 16%, P = .04). Geographic tongue was uncommon in both the study and the control group (4% vs 2%, P = .68). In the psoriasis group, patients with fissured tongue had a higher prevalence of cardiovascular disease (23.1% vs 4.9%), diabetes mellitus (28.2% vs 8.2%), and psoriatic arthritis (15.4% vs 1.6%) than those without this condition. Periodontitis was also associated with a higher prevalence of cardiovascular disease (28.6% vs 5.6%). Type of psoriasis, location, and time since onset were not significantly associated with oral lesions. Patients with oral lesions, however, had more severe disease (Psoriasis Area Severity Index [PASI], 3.9 vs 2.4; P = .05). Mean PASI was also higher in patients with fissured tongue (4.7 vs. 2.7, P = .03) and periodontitis (5.1 vs. 2.9, P = .04).ConclusionsThe prevalence of oral lesions, especially fissured tongue and periodontitis, is higher in patients with psoriasis than in healthy controls. Oral lesions were associated with more severe psoriasis and a higher prevalence of associated comorbidities. We recommend examining the oral cavity of patients with psoriasis, especially those with more severe disease and comorbidities, irrespective of type of psoriasis, location, or time since onset.     

Manifestaciones orales en pacientes con psoriasis. Prevalencia y asociación con sus características clínicas y epidemiológicas

Background and objectivePsoriasis is a multisystem disease associated with an increased prevalence of oral lesions. The aim of this study was to determine the prevalence of oral lesions in patients with psoriasis and examine associations with clinical and patient characteristics.Material and methodsWe conducted a cross-sectional study of patients with psoriasis and healthy controls seen between December 2019 and February 2020. We recorded biometric data, comorbidities associated with psoriasis, oral examination findings, and clinical characteristics of psoriasis.ResultsWe studied 100 patients with psoriasis and 100 controls. Oral lesions were more common in the psoriasis group (74% vs 46%, P < .001). The most common lesions were fissured tongue (39% vs 16%, P < .001) and periodontitis (28% vs 16%, P = .04). Geographic tongue was uncommon in both the study and the control group (4% vs 2%, P = .68). In the psoriasis group, patients with fissured tongue had a higher prevalence of cardiovascular disease (23.1% vs 4.9%), diabetes mellitus (28.2% vs 8.2%), and psoriatic arthritis (15.4% vs 1.6%) than those without this condition. Periodontitis was also associated with a higher prevalence of cardiovascular disease (28.6% vs 5.6%). Type of psoriasis, location, and time since onset were not significantly associated with oral lesions. Patients with oral lesions, however, had more severe disease (Psoriasis Area Severity Index [PASI], 3.9 vs 2.4; P = .05). Mean PASI was also higher in patients with fissured tongue (4.7 vs. 2.7, P = .03) and periodontitis (5.1 vs. 2.9, P = .04).ConclusionsThe prevalence of oral lesions, especially fissured tongue and periodontitis, is higher in patients with psoriasis than in healthy controls. Oral lesions were associated with more severe psoriasis and a higher prevalence of associated comorbidities. We recommend examining the oral cavity of patients with psoriasis, especially those with more severe disease and comorbidities, irrespective of type of psoriasis, location, or time since onset.     

Intestinal microbiome characterization of adult Brazilian men with psoriasis compared to omnivore and vegetarian controls

BackgroundPsoriasis is a chronic inflammatory disease associated with systemic inflammation and comorbidities. Changes in the composition of the intestinal microbiome are involved in the pathogenesis of inflammatory diseases and metabolic syndrome. Characterizing the intestinal microbiome of patients with psoriasis may be relevant for the understanding of its clinical course and comorbidity prevention.ObjectiveTo characterize the intestinal microbiome of men with psoriasis compared to omnivore and vegetarian controls (without psoriasis).MethodCross-sectional study of 42 adult males: 21 omnivores with psoriasis; and controls: 14 omnivores and 7 vegetarian individuals. The characterization of the intestinal microbiome was performed by metagenomic analysis. Serum levels of lipopolysaccharide-binding protein (LPB) and C-reactive protein (CRP) were evaluated.ResultsThe groups differed from each other regarding nutritional aspects and microbiome; individuals with psoriasis had a higher consumption of protein and lower consumption of fibers. Levels of LPB, CRP, and the Firmicutes/Bacteroidetes ratio were higher in the group with psoriasis than in the vegetarian group (p < 0.05). The genera Prevotella, Mogibacterium, Dorea, Bifidobacterium and Coprococcus, differed in the group with psoriasis compared to vegetarians; the genera Mogibacterium, Collinsella and Desulfovibrio differed from omnivores. A microbiome pattern linked to psoriasis (plsPSO) was identified, which was associated with higher LPB levels (rho = 0.39; p = 0.02), and lower dietary fiber intake (rho = −0.71; p < 0.01).Study limitationsOnly adult men were evaluated.ConclusionA difference was identified in the intestinal microbiome of adult men with psoriasis when compared to healthy omnivores and vegetarian controls. The identified microbiome pattern was correlated with dietary fiber intake and serum levels of LPB.     

Serum procalcitonin and C-reactive protein in the evaluation of bacterial infection in generalized pustular psoriasis

BackgroundThere is an obvious need for more prompt and specific biomarkers of bacterial infections in generalized pustular psoriasis patients.ObjectiveThe aim of this study was to evaluate the diagnostic properties and define appropriate cut-off values of procalcitonin and C-reactive protein in predicting bacterial infection in generalized pustular psoriasis patients.MethodsSixty-four generalized pustular psoriasis patients hospitalized from June 2014 to May 2017 were included in this retrospective study. The values of procalcitonin, C-reactive protein, details of infection, and other clinical parameters were analyzed.ResultsReceiver operating characteristic curve analysis generated similar areas (p = 0.051) under the curve for procalcitonin 0.896 (95% CI 0.782–1.000) and C-reactive protein 0.748 (95% CI 0.613–0.883). A cut-off value of 1.50 ng/mL for procalcitonin and 46.75 mg/dL for C-reactive protein gave the best combination of sensitivity (75.0% for procalcitonin, 91.7% for C-reactive protein) and specificity (100% for procalcitonin, 53.8% for C-reactive protein). Procalcitonin was significantly positively correlated with C-reactive protein levels both in the infected (r = 0.843, p = 0.040) and non-infected group (r = 0.799, p = 0.000).Study limitationsThe sample size and the retrospective design are limitations.ConclusionsThe serum levels of procalcitonin and C-reactive protein performed equally well to differentiate bacterial infection from non-infection in generalized pustular psoriasis patients. The reference value of procalcitonin and C-reactive protein applied to predicting bacterial infection in most clinical cases may not be suitable for generalized pustular psoriasis patients. C-reactive protein had better diagnostic sensitivity than procalcitonin; however, the specificity of procalcitonin was superior to that of C-reactive protein.     

Association of CYP2J2 polymorphism with susceptibility to psoriasis in Turkish population: a case–control study

BackgroundCytochrome P450 2J2 is mostly expressed in extrahepatic tissues; it metabolizes arachidonic acid to epoxyeicosatrienoic acids, with various cardio protective and anti-inflammatory effects. CYP2J2 polymorphism has been identified as a risk factor for cardiovascular diseases, but its association with psoriasis remains unknown.ObjectiveTo evaluate CYP2J2 polymorphism as a risk factor for psoriasis in the Turkish population.MethodsThere were 94 patients with psoriasis and 100 age- and sex-matched healthy controls included in the study. Detailed demographic and clinical characteristics were recorded, and Psoriasis Area and Severity Index (PASI) scores were calculated for psoriasis patients. Venous blood samples were collected from all the participants and CYP2J2 50G>T (rs890293) polymorphism was analyzed using polymerase chain reaction (PCR).ResultsBoth T allele and TT + GT genotype frequencies were increased in psoriasis vulgaris patients compared to the control group (p = 0.024 and p = 0.029 respectively, OR = 2.82, 95% CI: 1.11–7.15) No association between CYP2J2 polymorphism and clinical features of psoriasis was identified.Study limitationsA limited number of patients were included in the study.ConclusionCYP2J2 50G>T (rs890293) polymorphism was associated with an increased risk for PsV in the Turkish population.     

The association of polymorphisms of the vitamin D receptor gene with psoriasis in the Han population of northeastern China

BackgroundThe vitamin D receptor (VDR) is a master regulator of epidermal barrier function, inflammation, stem-cell proliferation, and microbial defense.ObjectiveTo evaluate the association between the VDR and psoriasis in the northeastern Chinese Han population.MethodsIn this case–control study, 342 patients with psoriasis and 341 controls were genotyped for five common VDR gene polymorphisms (ApaI, TaqI, BsmI, FokI, and Cdx2) by the Multiplex SNapSHOT method.ResultsThe frequency of ApaI (rs7975232) allele A was significantly increased in psoriasis relative to the control group (27.8% vs. 22.1%, p = 0.018); the allele A of the ApaI polymorphism showed a 1.35-fold increased risk of developing psoriasis. Haplotype analyses showed the BsmI/ApaI/TaqI/Cdx2/FokI GATGC to be significantly over-represented in psoriasis patients compared with controls (p = 0.012). The BsmI/ApaI/TaqI haplotype GCT was presented to a lesser extent in psoriasis patients in comparison with control patients (72.2% vs. 77.9%, p = 0.012).ConclusionsThese data suggest that VDR polymorphisms are associated with psoriasis in Northeastern Han Chinese population.     

Levels of the interleukins 17A, 22, and 23 and the S100 protein family in the gingival crevicular fluid of psoriatic patients with or without periodontitis

BackgroundPsoriasis and periodontitis are immunologically mediated chronic inflammatory diseases. Epidemiologic evidence has linked both; however, the change of markers in gingival crevicular fluid has been poorly evaluated.ObjectiveTo evaluate the levels of IL-17A, IL-22, IL-23, S100A7, S100A8, and S100A9 in gingival crevicular fluid of psoriatic and healthy subjects with and without periodontitis and their relations to psoriasis severity.MethodsCross-sectional study. Sample comprised the following groups: healthy controls without periodontitis or with mild periodontitis (n = 21), healthy controls with moderate or severe periodontitis (n = 18), individuals with psoriasis without or mild periodontitis (n = 11), and individuals with psoriasis and moderate or severe periodontitis (n = 32). Levels of IL-17A, IL-22, IL-23, S100A8, and S100A9 were determined by multiplex assay and S100A7 was measured by ELISA.ResultsNo inter-group differences in the levels of IL-17A, IL-22, IL-23, and S100A7 were found. S100A8 levels were higher in psoriatic patients than controls (p < 0.05). S100A8 was positively correlated with psoriasis severity in the group with psoriasis (p < 0.05). S100A9 exceeded the detection limits.Study limitationsThis pilot study presents a small sample size.ConclusionsThe concentrations of S100A8 were highest in psoriatic patients regardless of periodontal health/status. S100A8 was associated with the severity of psoriasis. The concentrations of interleukins and S100A7 were similar in psoriatic patients with or without periodontitis vs. healthy controls.     

Evaluation of monocyte to high-density lipoprotein ratio,lymphocytes, monocytes,and platelets in psoriasis

BackgroundPsoriasis is a chronic immune-mediated inflammatory skin disease that is associated with cardiovascular comorbidities.ObjectivesThe objective of this retrospective study is to assess the C-reactive protein, monocyte-to-high-density-lipoprotein ratio, neutrophil-to-lymphocyte ratio, platelet-to-lymphocyte ratio, and monocyte-to-lymphocyte ratio as inflammatory markers in patients with psoriasis and to search for a relationship between these parameters and psoriasis severity, as defined by the psoriasis area and severity index.MethodsThere were 94 patients with psoriasis and 118 healthy controls enrolled in the study. The C-reactive protein, monocyte-to-high-density-lipoprotein ratio, neutrophil-to-lymphocyte ratio, platelet-to-lymphocyte ratio, and monocyte-to-lymphocyte ratio values of two groups were retrospectively evaluated.ResultsStatistically significant differences were observed in terms of C-reactive protein, monocyte-to-high-density-lipoprotein ratio, neutrophil-to-lymphocyte ratio and monocyte-to-lymphocyte ratio between the patient and control groups (p = 0.001, p = 0.003, p = 0.038, and p = 0.007, respectively). Positive correlations were found between the psoriasis area and severity index and the values of C-reactive protein, monocyte-to-high-density-lipoprotein ratio, neutrophil-to-lymphocyte ratio, platelet-to-lymphocyte ratio, and monocyte-to-lymphocyte ratio (r: 0.381; p < 0.01, r: 0.203; p < 0.05, r: 0.268; p < 0.01, r: 0.374; p < 0.01, r: 0.294; p < 0.01, respectively).Study limitationsThe small sample size and the retrospective design of the study are limitations.ConclusionElevated C-reactive protein, monocyte-to-high-density-lipoprotein ratio, neutrophil-to-lymphocyte ratio, and monocyte-to-lymphocyte ratio were significantly associated with psoriasis. A positive correlation between C-reactive protein and monocyte-to-high-density-lipoprotein ratio leads to the suggestion that monocyte-to-high-density-lipoprotein ratio might be a reliable parameter in psoriasis during the follow-up. The relationship between the diasease and inflammatory parameters might provide early detection of cardiovascular morbidities in psoriasis patients.     

Association of MIF promoter polymorphisms with psoriasis in a Han population in northeastern China

BackgroundMacrophage migration inhibitory factor (MIF) is a pro-inflammatory cytokine that plays an important part in the pathogenesis of autoimmune diseases. A high level of MIF has been detected in plaques of psoriasis and the sera of patients with psoriasis. Polymorphisms associated with autoimmune and inflammatory diseases exist in the promoter region of MIF and alter its expression.ObjectiveThe aim of this study was to evaluate the potential relationship between functional polymorphisms of MIF and psoriasis in a Han population in northeastern China.MethodsTwo-hundred-and-forty psoriasis patients and a control group of 269 healthy volunteers were included in this study. We genotyped MIF-173G/C using polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP). MIF-794CATT_5–8 microsatellite polymorphism was genotyped by polyacrylamide gel electrophoresis (PAGE).ResultsNo significant difference in the distributions of alleles, genotypes and haplotypes was observed between patients and controls. When patients were divided into subtypes according to sex, family history and age of onset, distribution of the MIF-173C allele between male and female patients was significantly different (P = 0.04). MIF-173C allelic distribution between late onset psoriasis patients and controls was also different (P = 0.02), as well as late onset patients and early onset subjects (P = 0.04).ConclusionsThese results suggested a preliminary association between the MIF-173C allele and male psoriasis and late onset psoriasis in the studied population. In addition, the distributions of the two polymorphisms in Asian populations were quite different from the other continental populations.     

Demodex folliculorum infestations in common facial dermatoses: acne vulgaris,rosacea, seborrheic dermatitis

BackgroundDemodex mites are found on the skin of many healthy individuals. Demodex mites in high densities are considered to play a pathogenic role.ObjectiveTo investigate the association between Demodex infestation and the three most common facial dermatoses: acne vulgaris, rosacea and seborrheic dermatitis.MethodsThis prospective, observational case-control study included 127 patients (43 with acne vulgaris, 43 with rosacea and 41 with seborrheic dermatitis) and 77 healthy controls. The presence of demodicosis was evaluated by standardized skin surface biopsy in both the patient and control groups.ResultsIn terms of gender and age, no significant difference was found between the patients and controls (p > 0.05). Demodex infestation rates were significantly higher in patients than in controls (p = 0.001). Demodex infestation rates were significantly higher in the rosacea group than acne vulgaris and seborrheic dermatitis groups and controls (p = 0.001; p = 0.024; p = 0.001, respectively). Demodex infestation was found to be significantly higher in the acne vulgaris and seborrheic dermatitis groups than in controls (p = 0.001 and p = 0.001, respectively). No difference was observed between the acne vulgaris and seborrheic dermatitis groups in terms of demodicosis (p = 0.294).Study limitationsSmall sample size is a limitation of the study. The lack of an objective scoring system in the diagnosis of Demodex infestation is another limitation.ConclusionThe findings of the present study emphasize that acne vulgaris, rosacea and seborrheic dermatitis are significantly associated with Demodex infestation. Standardized skin surface biopsy is a practical tool in the determination of Demodex infestation.     

[Translated article] Association Between Demodex Infestation and Severe Acne Vulgaris: A Cross-Sectional Study of 168 Patients

Background and objectivesInfestation with Demodex mites has been associated with acne vulgaris. The aim of this study was to explore the association between Demodex infestation and severe acne vulgaris in outpatients seen at Hospital Regional Lambayeque in Chiclayo, Peru.Material and methodsWe conducted a cross-sectional study of 46 patients with severe acne and 92 patients with nonsevere acne. Severe acne vulgaris was diagnosed if the score was 3 or more on the Spanish Acne Severity Scale (EGAE, in its Spanish acronym). Demodex infestation was diagnosed when a skin surface biopsy showed more than 5 mites/cm².ResultsThe patients had a median age of 18 years (interquartile range, 15–20 years), 60.9% were male, 81.9% lived in an urban area, and 29.7% were infested with Demodex mites. In the bivariate analysis, severe acne vulgaris was significantly associated with Demodex infestation (P = .001), sex (P = .003), residence (P = .015), a paternal history of acne (P = .045), a maternal history of acne (P = .045), and type of skin (P < .001). In the multivariate analysis, after adjustment for male sex, urban residence, previous treatment, maternal and paternal history of acne vulgaris, and an oily skin type, patients with Demodex infestation were 4.2 times more likely to have severe acne vulgaris (95% CI: 1.6–10.9, P = .003).ConclusionDemodex infestation was associated with severe acne vulgaris in outpatients at our hospital.     

Autologous fat grafting for the treatment of sclerotic lesions and scars

IntroductionMicrofat grafting is a well-known technique that is underutilized in dermatology. Instead of removing sclerotic tissue, microfat grafting preserves the tissue and uses stem cells for remodeling its structure into normal tissue. We performed a retrospective study of patients treated with microfat grafting for sclerotic and atrophic skin lesions and scars.Patients and methodsSeventy-two microfat grafts were performed using the Magalon technique under general anaesthesia for the treatment of sclerotic and atrophic skin lesions. We performed grafts for different indications, such as scars (n = 55) and sclerotic and atrophic skin lesions (n = 17: Parry–Romberg syndrome, morphea). The main outcome was assessed for satisfaction during follow-up. In addition, an independent committee judged the results based on photographs.ResultsSatisfaction levels (e.g. results were judged to be “good”) were almost 91% (n = 50/55) for scars and 100% (n = 17/17) for atrophic and sclerotic skin lesions. Satisfaction levels according to the independent committee were 94.1% for sclerotic and atrophic lesions and nearly 51% for scars.ConclusionSatisfaction was high after microfat grafting for atrophic and sclerotic skin lesions. Microfat grafting enabled restoration of the skin texture by exploiting stem cell properties. It is an efficient dermatological therapy for sclerotic and atrophic lesions, for which there are few alternative treatments.     

Asociación entre la infestación por el Demodex sp. y el acné vulgar grave. Estudio transversal de 168 pacientes

Background and objectivesInfestation with Demodex mites has been associated with acne vulgaris. The aim of this study was to explore the association between Demodex infestation and severe acne vulgaris in outpatients seen at Hospital Regional Lambayeque in Chiclayo, Peru.Material and methodsWe conducted a cross-sectional study of 46 patients with severe acne and 92 patients with nonsevere acne. Severe acne vulgaris was diagnosed if the score was 3 or more on the Spanish Acne Severity Scale (EGAE, in its Spanish acronym). Demodex infestation was diagnosed when a skin surface biopsy showed more than 5 mites/cm².ResultsThe patients had a median age of 18 years (interquartile range, 15-20 years), 60.9% were male, 81.9% lived in an urban area, and 29.7% were infested with Demodex mites. In the bivariate analysis, severe acne vulgaris was significantly associated with Demodex infestation (P = .001), sex (P = .003), residence (P = .015), a paternal history of acne (P = .045), a maternal history of acne (P = .045), and type of skin (P < .001). In the multivariate analysis, after adjustment for male sex, urban residence, previous treatment, maternal and paternal history of acne vulgaris, and an oily skin type, patients with Demodex infestation were 4.2 times more likely to have severe acne vulgaris (95% CI: 1.6-10.9, P = .003).ConclusionDemodex infestation was associated with severe acne vulgaris in outpatients at our hospital.     

Serum granulysin as a possible key marker of the activity of alopecia areata

BackgroundAlopecia areata (AA) is an organ-restricted autoimmune condition of the hair follicles (HFs) that presents as nonscarring hair loss. A collapse of immunoprivilege for cell-mediated cytotoxicity and following attacks by cytotoxic T cells to anagen HFs are considered to play a major role in the pathogenesis of AA. However, there has been no useful marker for the activity of AA to date.ObjectiveThe aim of this study is to examine whether granulysin, which is known to reflect the activity of cytotoxic immune responses, is related to the disease activity of AA.MethodsWe evaluated serum granulysin levels in acute and chronic AA patients compared to healthy controls in the perspective of bald skin areas, prognosis, and co-existence of other allergic diseases. In addition, immunohistochemical analysis for granulysin-, CD4-, CD8-, and CD56-positive cells in the lesional skin of acute and chronic AA patients was performed.ResultsSerum granulysin levels were significantly elevated in both acute and chronic AA patients (p = 0.00081 and p = 0.0012, respectively). Intriguingly, serum granulysin levels were significantly associated with the broader bald skin areas (Spearman's r = 0.59, p = 0.017), and poorer prognosis in acute AA patients (p = 0.0080). They were also associated with co-existence of allergic disorders in AA patients (p = 0.026). Immunohistochemical staining demonstrated that perifollicular granulysin-bearing cells were mainly detected in acute AA lesions with dense lymphocytic infiltration, and that these granulysin-bearing cells were consistent with CD8⁺ T cells.ConclusionThe serum granulysin level may be a useful and novel marker for the disease activity in the acute phase of AA.     

Clinical Unmet Needs and Treatment Patterns of Paediatric Psoriasis Patients: A Real-World Evidence Study in Spain

Background and objectiveReal-world evidence of paediatric psoriasis (PsO) is lacking in Spain. The purpose of this study was to identify physician-reported disease burden and current treatment patterns in a real-world paediatric PsO patient cohort in Spain. This will enhance our understanding of the disease and contribute to the development of regional guidelines.Material and methodThis retrospective analysis of a cross-sectional market research survey assessed the clinical unmet needs and treatment patterns in patients with paediatric PsO in Spain, as reported by their primary care and specialist physicians, using data collected as part of the Adelphi Real World Paediatric PsO Disease-Specific Program (DSP™) between February and October 2020.ResultsSurvey data from 57 treating physicians were included (71.9% [N = 41] dermatologists, 17.6% [N = 10] general practitioners/primary care physicians, and 10.5% [N = 6] paediatricians); the final analysis included 378 patients. At sampling, 84.1% (318/378) of patients had mild disease, 15.3% (58/378) had moderate disease and 0.5% (2/378) had severe disease. Retrospectively reported physician-judged severity at the time of PsO diagnosis recorded 41.8% (158/378) of patients with mild disease, 51.3% (194/378) with moderate disease and 6.9% (26/378) with severe disease. Overall, 89.3% (335/375) of patients were currently receiving topical PsO therapy, while 8.8% (33/375), 10.4% (39/375) and 14.9% (56/375) of patients were currently receiving phototherapy, conventional systemics and biologics, respectively.ConclusionsThese real-world data reflect the current burden and treatment landscape of paediatric PsO in Spain. The management of patients with paediatric PsO could be improved by further educating healthcare professionals and developing regional guidelines.     

Pénfigo vulgar: estudio de cohorte retrospectivo de sus características clínicas,tratamientos empleados y evolución

BackgroundPemphigus vulgaris (PV) is an uncommon, serious disease that is treated with systemic corticosteroids and corticosteroid-sparing agents.ObjectivesTo describe and analyze the demographic and clinical characteristics of patients with PV.Material and methodsRetrospective cohort study of adults diagnosed with PV over a period of 12 years.ResultsPV presented with mucosal lesions in 20 of the 32 patients studied (63%); the most common site was the oral mucosa followed by the vulva. Mucosal involvement was more common in women (P = .03). Lesions were found at more than 1 mucosal site in patients whose disease began in the mucosa, independently of age or sex (P = .003). Disease onset before the age of 40 years was associated with generalized skin lesions (P = .003), a need for corticosteroid-sparing therapy (P = .05), and refractory PV (P = .02). Azathioprine was the most widely prescribed corticosteroid-sparing agent (in 22 patients). Eight patients (25%) were dependent on corticosteroids and disease recurred in 26 (81%). Complete remission, with or without treatment, was achieved in 15 patients (47%). Patients remained disease-free for a median of 14 months, and 2 patients died (6%).ConclusionOnset before the age of 40 years could be a sign of poor prognosis in patients with PV, as it was significantly associated with a higher risk of generalized skin involvement, a need for corticosteroid-sparing therapy, and refractory disease.