Juvenile Dermatomyositis: A Case Report and Review of Literature

Gupta, Pooja; Sharma, Swati; Chaudhary, Vijay Shanker; Khullar, Geeti; Siraj, Fouzia

doi:10.7759/cureus.3935

Cited by 6 publications

(5 citation statements)

References 17 publications

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“…Juvenile dermatomyositis (JDM) is an inflammatory myopathy affecting striated muscle, skin, and the gastrointestinal tract with an incidence of 3.2 cases per million children ( 1 ). Patients experience proximal muscle weakness and exhibit characteristic skin findings including heliotrope rash, Gottron papules, calcinosis cutis, and periungual erythema & telangiectasias ( 2 ). Before the use of steroid therapy, JDM had a mortality rate of 40%.…”

Section: Introductionmentioning

confidence: 99%

“…The etiology of JDM is not well understood, but it has been hypothesized that it involves a combination of environmental triggers, immune dysfunction, and specific tissue responses ( 2 ) with contributions from both defective adaptive and innate immune responses. Approximately 65% of JDM patients have myositis-specific or myositis-associated antibodies ( 3 , 5 ), and B cell depletion with rituximab improves symptoms in some patients ( 6 , 7 ) implicating a potential role for B cells.…”

Section: Introductionmentioning

confidence: 99%

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Expansion of a novel population of NK cells with low ribosome expression in juvenile dermatomyositis

Hilliard

Throm²,

Pingel³

et al. 2022

Front. Immunol.

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Juvenile dermatomyositis (JDM) is a pediatric autoimmune disease associated with characteristic rash and proximal muscle weakness. To gain insight into differential lymphocyte gene expression in JDM, peripheral blood mononuclear cells from 4 new-onset JDM patients and 4 healthy controls were sorted into highly enriched lymphocyte populations for RNAseq analysis. NK cells from JDM patients had substantially greater differentially expressed genes (273) than T (57) and B (33) cells. Upregulated genes were associated with the innate immune response and cell cycle, while downregulated genes were associated with decreased ribosomal RNA. Suppressed ribosomal RNA in JDM NK cells was validated by measuring transcription and phosphorylation levels. We confirmed a population of low ribosome expressing NK cells in healthy adults and children. This population of low ribosome NK cells was substantially expanded in 6 treatment-naïve JDM patients and was associated with decreased NK cell degranulation. The enrichment of this NK low ribosome population was completely abrogated in JDM patients with quiescent disease. Together, these data suggest NK cells are highly activated in new-onset JDM patients with an increased population of low ribosome expressing NK cells, which correlates with decreased NK cell function and resolved with control of active disease.

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Expansion of a novel population of NK cells with low ribosome expression in juvenile dermatomyositis

Hilliard

Throm²,

Pingel³

et al. 2022

Front. Immunol.

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“…Aberrant ossification has also been observed in conditions treated with prostheses such as breast augmentation, [33] hip arthroplasty, [34] hyoid suspension, [35] temporomandibular joint reconstruction, [36] and tibiofibular syndesmosis, [37] and even in autoimmune diseases such as dermatomyositis, [38] systemic sclerosis, [39] and giant cell arteritis [40] . Foreign body giant cells have been observed in these autoimmune diseases, although not simultaneously with ossification [41–43] …”

Section: Discussionmentioning

confidence: 99%

“…[40] Foreign body giant cells have been observed in these autoimmune diseases, although not simultaneously with ossification. [41][42][43] Our comprehensive literature review revealed that aberrant ossification (metaplastic or heterotopic) occurred in various lesions or conditions with potential for foreign body reactions (Table 1). These findings suggest that aberrant ossification is closely related to foreign body reactions.…”

Section: Discussionmentioning

confidence: 99%

Ossifying pilomatricoma and a novel hypothesis for its pathogenesis

et al. 2022

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Rationale:Pilomatricoma is a benign skin appendageal tumor derived from hair follicle matrix cells that commonly affects the head, neck, and upper extremities of the pediatric population. Since the original tumor description, diverse variants have been reported in the literature. Pilomatricoma with florid osseous metaplasia is described as an ossifying pilomatricoma and is recognized as a distinct variant of this benign tumor. However, the pathogenesis of this variant remains unclear. In this study, we present an uncommon case of ossifying pilomatricoma and address the pathogenesis of metaplastic ossification through a comprehensive literature review.Patient concerns:A 14-year-old boy presented with an asymptomatic protuberant mass in the preauricular region.Diagnosis:Based on its clinicopathological features, we diagnosed the lesion as an ossifying pilomatricoma.Interventions and outcomes:The lesion was surgically removed under local anesthesia. The postoperative course was uneventful during the 6-month postoperative follow-up.Lessons:We suggest that metaplastic ossification in ossifying pilomatricoma represents another feature of foreign body reaction to keratinous materials containing shadow cells in old lesions and a walling-off phenomenon to prevent exposure of surrounding tissues to keratinous materials.

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“…Su propuesta en relación con la RSE se encuentra estrechamente ligada a este concepto y busca la legitimización del negocio por parte de la sociedad. Según esta teoría, la sostenibilidad y el éxito empresarial dependen no sólo del logro de metas económicas, sino de la satisfacción de las necesidades de los principales stakeholders de la organización (Pirsch;Gupta;Grau, 2007).…”

Section: Universidad Y Stakeholdersunclassified

Impacto de la comunicación de la responsabilidad social universitaria en la selección de universidad y efecto mediador de la reputación

Erazo-Coronado¹,

Aristizábal²,

Garcés-Prettel

et al. 2020

EPI

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The emerging concept of university social responsibility (USR) consists in the fulfillment of the mission of the university in terms of ethical standards, community support, respect for human beings, and respect for the environment. Thus, universities must not only provide professional training but also instill ethical principles in students to allow them to behave as good citizens and help solve community problems. On the other hand, competition for students requires efficient management of university reputation and communication with their stakeholders, to demonstrate the accomplishment of the university’s mission and attract potential students. The objective of this paper is thus to explain the causal relationship between communication about university social responsibility (USR) activities through institutional websites and university selection, as well as the role of reputation. Research work was carried out based on an explanatory cross-sectional design, with a sample of 356 subjects from a population of 11th-grade students from public and private schools in the City of Barranquilla (Colombia). The results revealed that USR communication through institutional websites has a significant influence on university selection, not only directly but also indirectly, through reputation as a mediating variable. These findings contribute at a theoretical level by providing empirical evidence to understand and explain the analyzed topic. Furthermore, the results provide useful information to evaluate USR disclosure and implement strategies to make progress on USR issues. Resumen El naciente concepto de responsabilidad social universitaria (RSU) consiste en el cumplimiento de la misión de la universidad dentro de unos estándares éticos, de apoyo a la comunidad, respeto al ser humano y al medio ambiente. Así, pues, la universidad no sólo debe impartir formación profesional, sino inculcar a sus estudiantes principios éticos que les permitan comportarse como buenos ciudadanos y contribuir a la solución de los problemas de la comunidad. Por otra parte, la competencia entre universidades exige una gestión eficiente de su reputación y de la comunicación con sus distintos stakeholders, para demostrar a la sociedad el cumplimiento de su misión social, y para atraer estudiantes potenciales hacia la institución. Por lo tanto, este artículo tiene como objetivo explicar la relación de causalidad entre la comunicación de las prácticas de RSU en los sitios web institucionales y la selección de universidad, y el papel mediador de la reputación en esta relación. Para lograrlo, se llevó a cabo una investigación cuantitativa de nivel explicativo experimental y diseño transversal, con una muestra conformada por 356 sujetos, de una población de estudiantes de grado 11º de colegios públicos y privados de la ciudad de Barranquilla (Colombia). Los resultados revelan que la comunicación de las prácticas de RSU en los sitios web institucionales ejerce una influencia significativa directa en la intención de selección de universidad, e indirecta, a través de la reputación como variable mediadora. La evidencia empírica aportada representa un avance teórico en la comprensión y explicación del fenómeno estudiado, e igualmente servirá para que las universidades evalúen si están comunicando adecuadamente su responsabilidad social y adopten estrategias para avanzar en este aspecto.

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Juvenile Dermatomyositis: A Case Report and Review of Literature

Cited by 6 publications

References 17 publications

Expansion of a novel population of NK cells with low ribosome expression in juvenile dermatomyositis

Expansion of a novel population of NK cells with low ribosome expression in juvenile dermatomyositis

Ossifying pilomatricoma and a novel hypothesis for its pathogenesis

Impacto de la comunicación de la responsabilidad social universitaria en la selección de universidad y efecto mediador de la reputación

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