Search

This article discusses a case of IgA vasculitis in a patient with ulcerative colitis who was undergoing treatment with vedolizumab. IgA vasculitis is a rare symptom associated with ulcerative colitis, and vedolizumab is an antibody used to treat inflammatory bowel diseases. The patient experienced pulmonary hemorrhage and purpura on her limbs, which were preceded by episodes of hemoptysis. The article suggests that the development of IgA vasculitis may have been triggered by a combination of factors, including the alteration of intestinal microbiota due to vedolizumab treatment and lower respiratory tract infection. The patient's symptoms improved with prednisolone treatment, and there was no recurrence of symptoms after discontinuing prednisolone. The article concludes by stating that further research is needed to determine if vedolizumab increases the risk of IgA vasculitis in patients with inflammatory bowel disease. [Extracted from the article]

The article discusses the radiological improvement of peripheral arthritis in a patient with psoriatic arthritis treated with deucravacitinib, highlighting the first reported case of such improvement. Topics include the efficacy of deucravacitinib in treating plaque psoriasis, its potential role in treating psoriatic arthritis, and the implications of TYK2 inhibition in joint inflammation and bone repair.

Background ZNF597, encoding a zinc-finger protein, is the human-specific maternally expressed imprinted gene located on 16p13.3. The parent-of-origin expression of ZNF597 is regulated by the ZNF597:TSS-DMR, of which only the paternal allele acquires methylation during postimplantation period. Overexpression of ZNF597 may contribute to some of the phenotypes associated with maternal uniparental disomy of chromosome 16 (UPD(16)mat), and some patients with UPD(16)mat presenting with Silver-Russell syndrome (SRS) phenotype have recently been reported. Methods A 6-year-old boy presented with prenatal growth restriction, macrocephaly at birth, forehead protrusion in infancy and clinodactyly of the fifth finger. Methylation, expression, microsatellite marker, single nucleotide polymorphism array and trio whole-exome sequencing analyses were conducted. Results Isolated hypomethylation of the ZNF597:TSS-DMR and subsequent loss of imprinting and overexpression of ZNF597 were confirmed in the patient. Epigenetic alterations, such as UPD including UPD(16) mat and other methylation defects, were excluded. Pathogenic sequence or copy number variants affecting his phenotypes were not identified, indicating that primary epimutation occurred postzygotically. Conclusion We report the first case of isolated ZNF597 imprinting defect, showing phenotypic overlap with SRS despite not satisfying the clinical SRS criteria. A novel imprinting disorder entity involving the ZNF597 imprinted domain can be speculated. [ABSTRACT FROM AUTHOR]

Purpose: The purpose of the present study was to evaluate the dosimetric benefits of the irregular surface compensator (ISC) technique for whole breast radiotherapy compared with the field-in-field (FIF) technique. Materials and Methods: Radiotherapy was planned using both techniques in 50 breast cancer patients (25 left sided and 25 right sided). The Eclipse treatment planning system (Varian Medical Systems) was used for dose calculations. For the FIF technique, subfields were added to the main fields to reduce hot and cold regions; for the ISC technique, the fluence editor application was used to extend the optimal fluence. Planning target volume dose, dose homogeneity index (DHI), maximum dose, ipsilateral lung, and heart doses for the left breast irradiation and monitor unit (MU) counts required for treatment were compared between the two techniques. Results: Compared with the FIF technique, the ISC technique significantly decreased DHI values and volumes receiving >105% of the prescription dose, and increased volumes receiving >95% of the dose and MU count (P < 0.01 for all comparisons). For the heart and ipsilateral lung, the FIF technique significantly reduced volumes receiving >5 Gy compared with the ISC technique (P < 0.01); however, volumes receiving >10, 20, and 30 Gy and the values of a mean dose did not differ significantly between the techniques (P > 0.05). Conclusions: The ISC technique is preferred over the FIF technique. [ABSTRACT FROM AUTHOR]

Angiogenesis is regarded as an essential step in supporting tumour growth and metastasis. In haematological malignancies, including cutaneous T-cell lymphoma (CTCL), angiogenesis is increased and serum levels of some pro-angiogenic markers are elevated. The aim of this study was to investigate expression levels of placental growth factor (PlGF) and vascular endothelial growth factor (VEGF)-A in lesional skin and sera in patients with CTCL, and to assess the association of these factors with development of CTCL. A further aim was to investigate the effect of PlGF on lymphoma cell growth in vivo using a tumour inoculation model. Expression of PlGF and VEGF-A were significantly elevated in CTCL skin. Tumour cells expressed PlGF in some cases. Serum PlGF levels were increased in patients with advanced CTCL and correlated with disease markers. Moreover, PlGF enhanced lymphoma cell growth in vivo through increasing tumour vasculature. These findings suggest that angiogenesis plays a role in the progression of CTCL and raises the possibility of using inhibitors of PlGF in CTCL therapy. [ABSTRACT FROM AUTHOR]

The article presents a study which aimed to investigate the quality of life (QoL) of Japanese patients with hidradenitis suppurativa (HS). It mentions that the study used the skin disease-specific QoL scale, Dermatology Life Quality Index (DLQI), and Short Form-36 health survey version 2, a comprehensive health-related QoL scale that is applicable to various types of diseases.

In terms of software, "continuous development" of the software is the best quality assurance. Continuous development requires either the original developer to continue development, or the next generation of developers to take over the development. It has been noted that when the original developer has used "open source," a development paradigm in which the source code is kept open to all, highly motivated and competent developers will participate in development. This makes it easier for a project to survive. However, at the same time, when the source code is opened to a broad audience a fork in the source code tends to occur. When this happens, those with a high level of competence and motivation abandon the development and go their separate ways. In such a situation, it is difficult for a project to survive. In the case of Denshin 8 go, a guardian for the source code was appointed, and the original developer and the guardians avoided this dilemma. Richard Stallman of the GNU project and Linus Torvalds of Linux project act as legitimate guardians to avoid fork. [ABSTRACT FROM AUTHOR]

Success in software development is not simply "a person" or "a team" succeeding in development, but rather the execution of a smooth "shift to the next-generation" of developers, who continuously upgrade a single software product over a long span of time. In this case, different generations of developers share the source code. This paper analyzes this process using "Denshin 8 go," Japanese free software as one example. Initially, the original creator, Ishioka developed Denshin 8 go single-handed as closed source software and succeeded in motivating users through frequent upgrades. Several years after the initial release of Denshin 8 go Ishioka lagged behind in much needed upgrades, but one user group continued to use Denshin 8 go and was eager to improve the software. He disclosed the source code to the group, which in turn carried forward the process of development. As this case shows, the viewpoint that alternate generation of developers outside of a single company is caused by success in software development is 0known as "open source" software development. However, the reverse is not necessarily true. That is, simply becoming open source does not guarantee successful development. [ABSTRACT FROM AUTHOR]

Angiogenesis is a crucial process in the growth and progression of cancer, correlating with the metastatic potential of tumour cells. Angiopoietins are ligands for the endothelium-specific tyrosine kinase Tie2 receptor, which comprise 4 structurally related proteins, termed angiopoietin (Ang)-l, Ang-2, Ang-3 and Ang-4. The roles of Ang-1 and Ang-2 have recently been clarified as crucial in angiogenesis. In this report, we measured serum Ang-1 and Ang-2 levels in patients with cutaneous T-cell lymphoma (CTCL). Serum levels of Ang-2, but not Ang-1, in patients with Sézary syndrome were significantly higher than those in patch mycosis fungoides (MF), plaque/tumour MF, and healthy controls. In patients with CTCL, serum Ang-2 correlated with disease activity. Moreover, the numbers of Ang-2+ cells in lesional skin of CTCL were significantly larger than those in normal skin. These results suggest that Ang-2 may have important roles in the development of CTCL. [ABSTRACT FROM AUTHOR]

Primary cutaneous follicular helper T (TFH)-cell lymphoma has recently been proposed, and is characterized by proliferation of malignant T cells expressing TFH-cell markers, such as CXCL13, accompanied by numerous reactive B cells. We report here a patient whose skin histology showed massive infiltration of both T and B cells, with a proliferation of arborizing high endothelial venules and follicular dendritic cells. Infiltrating T cells were positive for CXCL13, programmed death (PD)-I, inducible T-cell co-stimulator, and BCL-6. Southern blot analyses using DNA from the skin revealed monoclonality of both T and B cells. The patient had marked resistance to treatments, and complete remission was achieved only after allogeneic stem cell transplantation. The present case showed overlapping features with angioimmunoblastic T-cell lymphoma (AITL), although systemic symptoms were not observed. Further study is needed to define the criteria of this provisional entity, representing the cutaneous counterpart of the nodal follicular peripheral T-cell lymphoma or AITL. [ABSTRACT FROM AUTHOR]

The article discusses hidradenitis suppurativa has a chronic, inflammatory, recurrent, debilitating disease of hair follicles with painful, deep-seated, inflamed lesions, Topics include the participants or legal guardians provided written informed consent in compliance with the principles of the Declaration of Helsinki; and the study has approved by the Medical Ethics Committee of Nihon University.

Cutaneous involvement is frequent in adult T-cell leukaemia/lymphoma (ATLL), a peripheral T-cell neoplasm caused by human T-cell lymphotropic virus type I (HTLV-I). Patients with ATLL manifest different types of skin lesions, including nodules, plaques, ulcers, erythroderma and purpura. It has been reported that the type of skin eruption is an independent prognostic factor for ATLL. We report here a rare case of a 62-year-old Japanese woman with smouldering-type ATLL, first manifested by lichen planus-like skin lesions on the lower leg. This case report highlights the multiplicity of skin manifestations in ATLL. [ABSTRACT FROM AUTHOR]

The article presents a study which examined the soluble form of herpesvirus entry mediator (sHVEM) receptor expression and its ligand, lymphotoxin-like expression (LIGHT), in patients with cutaneous T-cell lymphoma (CTCL). The sHVEM, serum interleukin-2R, interleukin-4 and interleukin-10 and eosinophil levels of patients with mycosis fungigoides and Sézary were measured. A higher level of sVEM and LIGHT levels was recorded.

The article discusses a study on temporal variation of pruritus in atopic dermatitis (AD) patients. Topics addressed include pruritus intensity in pruritic dermatoses patients, sensitive chemokines associated with pruritus changes, and chemokines' serum cytokine levels. Also mentioned are the expression of Th1 chemokines in response to Th2-mediated inflammation and the use of visual analogue scale to assess pruritus intensity.

The article describes the case of a 60-year-old woman diagnosed with psoriasis who developed exacerbation of mycosis fungoides (MF) with large cell transformation while receiving treatment with infliximab. Topics discussed include the samples of tumour necrosis factor inhibitors used for patients with psoriasis, the results of skin biopsy and histological examination done on the patient and the administration of topical corticosteroids and oral etoposide.