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Several observational studies have assessed the correlation between Merkel cell carcinoma and Merkel cell polyomavirus with variable results. The objective of this systematic review was to determine whether there is a correlation between Merkel cell carcinoma and Merkel cell polyomavirus. Studies assessing the relationship between Merkel cell carcinoma and Merkel cell polyomavirus from January 2008 to August 2014 were pooled from Medline, Embase, PubMed, Cochrane Database of Systemic Reviews and Google Scholar. From each study we collected the first author's last name, publication year, country of origin, type of study design, characteristics of participants, possible variables incorporated into the multivariable analyses and the risk ratio (RR) for Merkel cell carcinoma associated with Merkel cell polyomavirus combined with the corresponding 95% confidence interval (CI). Methodological assessment of the study was evaluated using the Newcastle-Ottawa scale. Crude RR was calculated from the data provided in each article. Meta-analyses for the global RR and for the proportion of positives in both case and control samples were performed. In addition, in order to explore the sources of heterogeneity among the studies, meta-regression and sensitivity analyses are also provided. A total of 22 studies were identified for the analysis. The pooled RR from random-effects analysis was determined to be 6.32 (95% CI, 4.02-9.93). Global proportions of positive samples were 0.79 (95% CI, 0.72-0.84) and 0.12 (95% CI, 0.08-0.19) in the case and control groups, respectively. The findings support the association between Merkel cell carcinoma and Merkel cell polyomavirus. However, a non-negligible percentage of positive results have been identified in controls. Some caution must be taken in the interpretation of these results because heterogeneity between studies was found., (© 2015 British Association of Dermatologists.)

Genetic factors are involved not only in the overall risk of suffering psoriasis, but also in their clinical characteristics and eventually in drug outcome. Biological therapies have dramatically improved the prognosis of Psoriasis. However, these treatments are very expensive and patients often exhibit a heterogeneous response that could be partially attributed to their genetic background. Thus, the research for genetic markers in psoriatic patients that could predict a poor response to biological therapies is an important issue. Our aim was to evaluate the effect of DNA variants at the "TNFα pathway" that could affect the risk of developing Psoriasis or the response to biological therapies among these patients. The genetic association study included a total of 518 Psoriatic patients and 480 healthy controls. Ninety of these patients received biological treatment and based on the change in the PASI score after 24 weeks were classified as good (PASI score ≥75%), intermediate (PASI 50-75), and non-responders (PASI <50). Next generation sequencing (NGS) with semiconductor-array technology was used to identify the nucleotide variants in the TNF α, TNFRSF1A and TNFRSF1B, and we only found three missense amino acid changes, all in TNFRSF1B. Interestingly, we found a significantly higher frequency of rs1061622 G carriers among CW6-positive patients (p = 0.004; OR = 1.69, 95% CI = 1.18-2.41). Allele G (p.196R) carriers were significantly more frequent in the non-responder group (56%) (p = 0.05). In conclusion, we report a significant association between the TNFRSF1B p.M196R variant and the risk for psoriasis and the response to treatment with anti-TNF or anti-Il-12/Il-23. The genotyping of this polymorphism could help to optimize the treatment by identifying patients with a likely poor response to biological drugs.

Our aim was to determine whether the HLA-Cw6 and late-cornified envelope (LCE) deletion polymorphisms were related to disease improvement among psoriasis patients treated with anti-tumor necrosis factor (TNF) antibodies. The study included a total of 116 patients. Positive response (68%) was defined as a reduction of at least 75% of the Psoriasis Area and Severity Index (PASI) after 24 weeks of starting the anti-TNF therapy. We found a trend toward a better response among Cw6-positive patients. The frequency of patients who did not reach the PASI75 was higher among the LCE-DD patients (P=0.028; odds ratio=2.45, 95% confidence interval=1.09-5.52). Patients who were Cw6-positive and LCE-I carriers (ID/II) were significantly more likely to reach PASI75 than those who were Cw6-negative and LCE-DD (P=0.034; odds ratio=3.14, 95% confidence interval=1.07-9.24). In conclusion, we found an interaction between the HLA-Cw6 and LCE genotypes on disease improvement among psoriatic patients treated with anti-TNFs.

Objective: To assess the efficacy and safety of adalimumab in biologic naïve versus non-biologic naïve psoriasis patients., Methods: A retrospective multicenter study of patients with moderate-to-severe psoriasis treated uninterruptedly with adalimumab between 6 and 36 months., Results: A total of 100 patients (52 men, 48 women; mean age 45.5 years; 90% plaque-type psoriasis) were included. Previous treatments included biologics in 53 and non-biologic modalities in 47. The mean (SD) start Psoriasis Area and Severity Index (PASI) score was 15.9 (11.1) (range 2-55). The median follow-up was 18 months (interquartile range 10-24 months). Sixteen weeks after the start of treatment, 94% of the patients presented PASI 75 response, 76% PASI 90, and 39% were in complete remission (PASI 100). Similar findings were obtained in the groups of biologic naïve and non-biologic naive patients. At the final visit, around 37% in all groups were in complete remission. At the end of the study, 74 patients continued treatment with adalimumab (43 [91.5%] in the biologic naïve group and 31 [58.5%] in the non-biologic naïve group, p<0.001)., Conclusion: This study confirms the efficacy and safety of adalimumab for moderate-to-severe psoriasis without differences between biologic naïve and non-naïve patients.

Bruton's tyrosine kinase (Btk) is a member of the Tec family of protein tyrosine kinases involved in B cell development and proliferation in neoplastic human lymphoid tissues. We used immunohistochemistry to evaluate a polyclonal anti-Btk antibody on formalin-fixed paraffin-embedded tissue blocks. The tested samples included normal lymphoid tissues, tissue samples of 395 different lymphomas and 14 malignant lymphoid cell lines. Btk was expressed more often in B cell lymphomas than in T cell lymphomas. This correlated well with the results obtained on B cell lymphoma cell lines, which strongly expressed Btk, in contrast to T cell lymphoma cell lines. More than 60% of myelomas expressed Btk. Among Hodgkin lymphomas, the nodular lymphocyte predominant variant was more often positive (14/16) than the classical variant (6/27). Only one out of three Hodgkin lymphoma-derived cell lines showed a few atypical large cells expressing Btk. Btk represents a useful marker to identify B cell non-Hodgkin lymphomas. Furthermore, Btk might help to distinguish the nodular lymphocyte predominant variant of Hodgkin lymphomas from the classical form. Finally, in view of the recently discovered therapeutic potential of Btk inhibitors in lymphoma, we report the pattern of expression of Btk in a large collection of different types of lymphoma.

Background: Psoriasis (Ps) has been associated with an increased incidence of cardiovascular disease. Interesting epidemiological evidence suggests associations between Ps and dyslipidemia (DL), a well-established risk factor for cardiovascular disease., Objective: This study aimed to investigate the association between Ps and multiple measurements of DL, which include levels of triglycerides (TGs), low-density lipoprotein (LDL) cholesterol, high-density lipoprotein (HDL) cholesterol and total cholesterol (TCh). We also studied the relationship between DL and disease duration., Methods: A prospective hospital-based study was conducted. A total of 661 Caucasian patients with chronic plaque Ps and 661 sex- and age-matched controls were enrolled., Results: Multivariate analysis showed that in psoriatic patients the odds ratio (OR) of TCh >200 mg/dl was 1.406 (95% confidence interval 1.115-1.173), the OR of LDL cholesterol >130 mg/dl was 1.375 (95% confidence interval 1.088-1.738), the OR of HDL cholesterol <40 mg/dl was 0.881 (95% confidence interval 0.599-1.297), and the OR of TGs >150 mg/dl was 1.041 (95% confidence interval 0.783-1.385). We did not find a relationship between lipid levels and disease duration., Conclusion: Based on our population Ps is associated with alterations in TCh and LDL cholesterol, but not in TGs and HDL cholesterol, when ATP III panel levels are used. These alterations are not related to disease duration., (© 2015 S. Karger AG, Basel.)

Background: Common DNA variants in IL12B, IL23R and IL23A have been associated with an increased susceptibility to psoriasis (Ps) and psoriatic arthritis (PsA). Metabolic comorbidities and cardiovascular risk factors have also been associated to both Ps and PsA., Objective: To analyze the effect of single nucleotide polymorphisms (SNPs) previously linked to Ps (IL12B rs6887695 and rs3212227, IL23R rs2201841 and rs11209026, and IL23Ars2066808) in the main phenotype and metabolic/cardiovascular characteristics among Ps patients from a Northern Spanish population., Methods: The aforementioned genetic variants were determined in a total of 405 chronic plaque Ps patients and 426 controls. Subsequent statistical analysis included stratification for psoriatic clinical characteristics (age of onset, disease severity, familial psoriasis, HLA-Cw6, nail psoriasis) plus diabetes mellitus type 2, arterial hypertension, dyslipidemia and ischemic cardiac events as comorbidities., Results: An association between IL23R rs11209026-GG genotype with a more severe disease (p=0.02, OR=2.11, 95% CI=1.13-3.95). Carriers of the IL23A rs2066808-A allele were significantly more frequent among PsA patients (p=0.016, OR= 3.04, 95% CI=1.19-7.78). We found significant associations between three SNP genotypes and type 2 diabetes: IL12B rs6887695-CC (p=0.03, OR=2.90, 95% CI=1.09-7.69), IL12B rs3212227-CC (p=0.035, OR=5.90, 95% CI= 1.35-25.73) and IL23R rs2201841-GG (p= 0.027, OR=2.69, 95% CI=1.09-6.66)., Conclusion: In our population, genetic variation at IL12B, IL23R and IL23A has an influence not only on the risk for Ps but also on disease severity and type 2 diabetes mellitus., (Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.)

Complete androgen insensitivity syndrome is an extremely infrequent disease. The patients exhibit female phenotype because of insensitivity to the androgen receptor and may develop tumors, especially in their undescended gonads. We report a case of bilateral Sertoli cell adenoma in gonads with unilateral serous cystadenoma, in an elderly phenotypic woman with primary amenorrhea. We also provide radiological and pathological studies.

A recent genomic survey identified the association between a common single nucleotide polymorphism (SNP) at the CARD14 gene (SNP rs11652075; p.Arg820Trp) and psoriasis (Psor). Our aim was to replicate the association between this polymorphism and to determine whether other CARD14 variants could explain the association. A total of 400 Psor patients (mean age 47±15; 55% male) and 420 healthy controls (mean age 51±16; 56% male) all Caucasian were genotyped for rs11652075. The rs11652075 CC genotype was significantly associated with Psor in our population (p=0.003; odds ratios=1.59; 95% confidence intervals=1.16-2.19; statistical power >80). The sequencing of the whole CARD14 coding exons in a total of 15 patients did not identify other DNA variants that could explain this association. We did not find significant differences (allele/genotype frequencies) between the patients according to disease severity, presence of arthritis, onset of age, and family history of Psor. We confirmed the association between SNP rs11652075 at the CARD14 gene and Psor. The absence of other coding variants among our patients supported a direct role for this missense polymorphism on Psor risk.

Several observational studies have assessed the association between psoriasis, psoriatic arthritis (PsA) and type 2 diabetes mellitus, with inconclusive results. We set out to investigate the association between psoriasis, PsA and type 2 diabetes mellitus. Observational studies assessing the relationship between psoriasis or PsA and type 2 diabetes mellitus up to December 2012 were identified by electronic and hand searches in Medline, Embase, PubMed, the Cochrane Database of Systematic Reviews and Google Scholar. For each study we collected the first author's last name, publication year, country of origin, study design, characteristics of participants (sample size, age and sex), the variables incorporated into the multivariable analyses, and the odds ratios (ORs) of psoriasis associated with diabetes along with the corresponding 95% confidence intervals (CIs). From the data provided in each article, the crude OR was also calculated. Forty-four observational studies (in 37 articles) were identified for the final analysis. The pooled OR from random-effects analysis was determined to be 1·76 (95% CI 1·59-1·96). The highest risk was for patients suffering from PsA (OR 2·18, 95% CI 1·36-3·50). We also observed a dose effect in the risk of suffering from type 2 diabetes mellitus, as patients considered as having severe psoriasis had higher risk (OR 2·10, 95% CI 1·73-2·55) than the pooled OR. We perform meta-regression and sensitivity analyses to explore sources of heterogeneity among the studies and to determine how they would influence the estimates, and found no significant influence in the results of the meta-analyses. The findings support the association between psoriasis, PsA and type 2 diabetes mellitus. Some caution must be taken in the interpretation of these results because there may be heterogeneity between studies., (© 2013 British Association of Dermatologists.)

Background and Aims: Recent genomic surveys have identified IL12B as susceptibility locus for psoriasis (Ps). Our aim was to replicate the association between IL12B SNPs and Ps. In addition, we sequenced the IL12B gene in several patients to identify new variants that could explain the disease-risk., Results: A total of 304 Ps-patients and 422 healthy controls (all Caucasian Spanish) were genotyped for three IL12B polymorphisms. SNP rs6887695 (GG genotype) was significantly associated with Ps (p=0.002; OR=1.60, 95% CI=1.19-2.16). This genotype was also more frequent among patients with severe psoriasis (p=0.03). Sequencing of 30 patients with the risk genotype identified several IL12B reported SNPs. Allele and genotype frequencies for two putative functional variants (rs3213120 and rs3213119) did not differ between patients and controls., Conclusions: Our study confirmed rs6887695 as a risk factor for Ps. No other IL12B variants that could explain this association were found in our patients., (Copyright © 2012 Elsevier Ltd. All rights reserved.)

Mitochondrial dysfunction could contribute to the pathogenesis of psoriasis (Ps) and Ps-arthritis (PsA). Several common mtDNA polymorphisms/haplogroups have been linked to differences in the production of reactive oxygen species and mitochondrial oxidative damage. To test the hypothesis of an association between mtDNA variants and Ps/PsA, we studied the single-nucleotide polymorphisms that define the common European haplogroups in a total of 325 patients and 300 controls from Spain. No allele/haplogroup was significantly associated with the risk for Ps. However, haplogroup J was significantly less frequent among patients with PsA, suggesting a protective effect in our population (p=0.04; odds ratio=0.39). We concluded that mtDNA may have a role in Ps and PsA.

Background:   Several studies stated that patient with psoriasis carried an increased risk of psoriasis but some studies did not demonstrate this association., Objectives:   The aim of this study was to evaluate the prevalence of hypertension in psoriasis based on a sample of Spanish population., Methods:   This was a hospital-based case-control study involving 661 psoriatic patients (cases) and 661 control matched by gender and age. Meta-analysis of the previous studies was made., Results:   The prevalence of hypertension was significantly higher in psoriasis patients than controls (30.3%, 21.3%, respectively, P < 0.001). In a multivariate analysis, hypertension was associated with psoriasis after controlling for age, gender, diabetes, obesity and smoking (OR = 1.44, 95% confidence interval: 1.07-1.94)., Conclusion:   The results of this study support the association between psoriasis and hypertension., (© 2011 The Authors. Journal of the European Academy of Dermatology and Venereology © 2011 European Academy of Dermatology and Venereology.)

There is accumulating evidence showing a relationship between psoriasis and an increased risk of developing cardiovascular risk factors, including diabetes mellitus type 2 and ischemic heart disease. Our aim was to investigate if there is any difference in the diabetes risk profile among psoriatic patients based on clinical findings. To test this, we carried out a prospective and descriptive hospital-based study. Our results suggest that the highest risk of suffering from diabetes mellitus type 2 among psoriatic patients is in patients suffering from non-familial and late-onset disease and in patients suffering from psoriatic arthritis., (© 2011 The Authors. Australasian Journal of Dermatology © 2011 The Australasian College of Dermatologists.)

Psoriatic patients have a higher prevalence of diabetes mellitus type 2 (DM). Since dipeptidyl peptidase IV (DPP-IV) dysregulation is present in DM and psoriasis, DPP-IV inhibitors have been proposed as therapeutic agents for both conditions. We report a psoriasiform eruption induced by sitagliptin, a DPP-IV inhibitor. The role of DPP-IV in the pathogenesis of DM is well established; however data on psoriatic patients is contradictory. More studies are required to elucidate the effect of DPP-IV inhibitors and their relationship with DM and psoriasis., (© 2011 The Authors. Australasian Journal of Dermatology © 2011 The Australasian College of Dermatologists.)

Nitric oxide (NO) is a potent regulator of keratinocyte growth and differentiation that has been implicated in the pathogenesis of psoriasis (Ps). The NOS3 -786 T/C (SNP id rs2070744; http://www.ensembl.org ), intron 4 variable number tandem repeat (VNTR), and Glu298Asp (SNP id rs1799983) polymorphisms, have been associated with differences in NO plasma concentrations and with the risk of hypertension (HT) and ischemic cardiac disease. The aim of this study was to determine whether the above-mentioned NOS3 variants contributed to the risk of Ps, and were associated with the risk for HT and CAD in these patients. A total of 368 patients with chronic plaque Ps and 400 healthy controls were genotyped for the NOS3 -786 T/C, intron 4 VNTR, and Glu298Asp polymorphisms. Carriers of the -786 C allele were significantly more frequent among the patients (p < 0.001). Carriers of the 4-repeats allele (45 + 44 genotypes) were also more frequent a (p < 0.001). No significant difference was found for the Glu298Asp polymorphism. None of the NOS3 variants was associated with Ht and CAD in our population. In conclusion, NOS3 gene polymorphism would be risk factors for developing Ps.

Background and Objectives: The nails are affected in a substantial number of patients with psoriasis. Nevertheless, few epidemiological studies have reported the characteristics of patients with nail psoriasis. Here we describe the epidemiology of nail psoriasis and the main characteristics of affected patients., Patients and Methods: We undertook a prospective case-control study at Hospital Universitario Marqués de Valdecilla and Hospital Universitario Central de Asturias in Spain between January 2007 and December 2009., Results: Of a total of 661 patients included, 47.4% were diagnosed with nail psoriasis, which was 13.5% more prevalent in men. The group of patients with nail disease had more severe psoriasis (12.82 vs 8.22 points on the psoriasis area and severity index) and a longer disease duration (20.30 vs 13.94years), and included a larger percentage of patients with psoriatic arthritis (29.7% vs 11.5%), a positive family history of the disease (53.7% vs 42.8%), and a body mass index greater than 30 (31.6% vs 23.9%). A larger percentage of the patients with nail disease had early-onset psoriasis (74.1% vs 65.5%) and fewer were carriers of the human lymphocyte antigen Cw*0602 allele (33% vs 50.3%)., Conclusions: Nail disease is frequent in psoriasis and is associated with greater severity of psoriasis and a larger number of comorbidities., (2010 Elsevier España, S.L. y AEDV. All rights reserved.)

The epidermal differentiation complex gene cluster is on chromosome region 1q21.3, where the PSORS4 locus was mapped. A common deletion of two LCE genes (LCE3C&#95;LCE3B-del) was associated with psoriasis. Individuals homozygous for the deletion have an impaired response of the skin barrier to exogenous agents, facilitating the systemic skin inflammation characteristic of psoriasis.

Cetuximab, an epidermal growth factor receptor inhibitor, is a chemotherapeutical agent featuring well-known adverse dermatological effects related to tumour-response prognosis. In psoriasis, the epidermal growth factor receptor is overexpressed; hence, the expectation would be that an epidermal growth factor receptor inhibitor could improve psoriatic lesions. We report a case of psoriasis induced by cetuximab, which was a surprising adverse effect., (© 2010 The Authors. Australasian Journal of Dermatology © 2010 The Australasian College of Dermatologists.)

Imiquimod is an immunomodulator of the imidazoquinoline group which possesses antiviral and antitumour activities. Although its mechanism of action has not been entirely elucidated yet, imiquimod 5% cream has been shown to be an efficient, long-lasting and safe therapy for multiple actinic keratoses in non-immunosuppressed patients and in transplant recipients. We report the case of a 44-year-old patient with a third renal transplant who developed an acute tubular necrosis confirmed by renal biopsy after the use of imiquimod 5% cream. The result of a literature search revealed a wide variety of side effects attributable to the use of imiquimod., (Copyright © 2011 S. Karger AG, Basel.)

Scalp lesions are common in psoriasis and difficult to treat. Scientific evidence on the topic is scant and fragmentary, especially with respect to long-term treatment. This consensus statement is based on a critical assessment of the results of a MEDLINE search for clinical trials of the efficacy and safety of therapies used to treat scalp psoriasis. The recommendations were developed by an expert panel using the Delphi process to reach a consensus and then ratified by the members of the Psoriasis Group of the Spanish Academy of Dermatology and Venereology. The recommended induction therapy for scalp psoriasis is either a topical corticosteroid or a topical treatment combining calcipotriol and betamethasone. The choice of an appropriate vehicle is crucial in improving effectiveness and patient adherence to treatment. The only formulations that have been studied in the long-term treatment of scalp psoriasis are a combination of calcipotriol and betamethasone in gel and calcipotriol alone in solution.

Objectives: Inflammation plays a major role in psoriasis (Ps). The variation at several genes that encode components of the inflammatory pathways have been linked to the risk for Ps. Our objective was to examine the association between Ps and three polymorphisms at the chemokine receptors CCR5 and CCR2., Methods: A total of 382 Ps patients and 500 healthy controls from Spain were genotyped for the CCR5-32bp deletion (DeltaCCR5), the rs1799988 (CCR5 promoter), and the CCR2-I64V (rs1799864) polymorphisms., Results: Allele and genotype frequencies did not differ between patients and controls for any of the three polymorphisms. However, the frequency of the CCR2-64I carriers was significantly higher in the patients who developed arthritis (n=81) compared to patients without arthritis (p=0.0007)., Conclusions: Our work suggests that the genetic variation at the CCR2/CCR5 genes did not contribute to the risk for Ps, but CCR2 polymorphisms could modulate the risk for arthritis in patients with psoriasis., (Copyright (c) 2010 Elsevier Ltd. All rights reserved.)

Anti-TNFs have a wide spectrum of skin lesions, psoriasis being found among them paradoxically. A 42-year old woman with a history of rheumatoid arthritis since 19 years of age was referred to the Dermatology service due to pustular psoriasis on both soles during treatment with etanercept. Due to her incapacity to walk and the pain reported by the patient, etanercept was replaced with adalimumab with clinical improvement and total disappearance of the lesions at six weeks of switching the anti-TNF. Tumor necrosis factor inhibitors are drugs that act in T cell mediated diseases. The appearance of psoriasiform rashes is more frequent than reported in the literature. They appear in all the indications and with all the anti-TNFs. Adalimumab is the most frequent. Three types of reactions are produced, the pustular one being the most frequent. It predominates in women and may appear chronologically at any time during the treatment. Regarding the treatment, it is recommendable to continue with the anti-TNFs.

Background: An association between a common deletion comprising the late cornified envelope LCE3B and LCE3C genes (LCE3C&#95;LCE3B-del) and Psoriasis (Ps) has been reported. The expression of these LCE genes was induced after skin barrier disruption and was also strong in psoriatic lesions. The damage to the skin barrier could trigger an epidermal response that includes the expression of genes involved in the formation of skin barrier., Methods: We determined the LCE3C&#95;LCE3B-del genotype in 405 Ps patients and 400 healthy controls from a Northern Spain region (Asturias). These patients and controls were also genotyped for the rs4112788 single nucleotide polymorphism, in strong linkage disequilibrium with the LCE3C&#95;B cluster. The LCE3B and LCE3C gene variant was determined in the patients through SSCA, DHPLC, and direct sequencing., Results: Allele and genotype frequencies did not differ between patients and controls for the rs4112788 and LCE3C&#95;LCE3B-del polymorphisms. However, del/del homozygotes were significantly higher among patients with chronic plaque type Ps who did not develop arthritis (p = 0.03; OR = 1.4; 95%CI = 1.03-1.92). The analysis of the coding sequence of LCE3B and LCE3C in the patients who had at least one copy of this showed that only one patient has a no previously reported LCE3B variant (R68C)., Conclusion: Our work suggested that homozygosity for a common LCE3C&#95;LCE3B deletion contributes to the risk of developing chronic plaque type Ps without psoriatic arthritis. Our work confirmed previous reports that described an association of this marker with only skin manifestations, and supported the concept of different genetic risk factors contributing to skin and joint disease.

Data on the potential influence of the angiotensin-converting enzyme (ACE) insertion/ deletion (I/D) genotype on psoriasis are contradictory. Our aim was to investigate the relationship between the ACE gene I/D polymorphism and psoriasis/psoriatic arthritis. To investigate this, a genetic association study was conducted among 268 unrelated patients with psoriasis and 272 controls. The distribution of DD, ID, and II genotypes did not significantly differ between psoriatic patients and controls (DD: 39.6% vs. 34.2%; ID: 46.3% vs. 53.3%; II: 14.1% vs. 12.5%). In conclusion, the ACE polymorphism is not likely to be associated with either psoriasis or psoriatic arthritis in this study.

Schönlein-Henoch purpura is a small vessel disease that affects mainly skin and kidney, although several gastrointestinal symptoms may occur including abdominal pain, intussusception, perforation or bleeding. Massive lower gastrointestinal haemorrhage is rare and even more as the main symptom of the disease. We present a case of a 2-year-old boy with Schönlein-Henoch purpura who developed a massive lower gastrointestinal bleeding requiring blood transfusion. In this patient both Schönlein-Henoch purpura and gastrointestinal haemorrhage were successfully treated with intravenous methylprednisolone, avoiding surgical intervention. Physicians need to have a high index of suspicion when evaluating these patients, even more when dermatologic signs are scarce. Glucocorticosteroid therapy may be effective when treating severe gastrointestinal symptoms.