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      Cracking the code: high ferritin load with Salmon-Colored skin episodes

      case-report

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          Abstract

          A 37-year-old previously healthy male presented to the Emergency Department with a two-week history of intermittent fevers, joint pain, sore throat, and a diffuse salmon-colored rash. Examination revealed a pruritic rash with joint swelling and red spots in the oropharynx. Initial sepsis management was instituted, but subsequent investigations, including infectious, hematologic, and autoimmune workups, were inconclusive. Notably, elevated ferritin levels prompted consideration of life-threatening conditions like Hemophagocytic Lymphohistiocytosis, which was ultimately ruled out. Adult-onset Still’s Disease (AOSD) emerged as the leading diagnosis following the exclusion of other potential causes. A skin biopsy was performed with non-specific findings and corticosteroid treatment led to significant improvement. This case illustrates the clinical decision-making process of diagnosing AOSD and highlights the potential utility of novel AI technology in dermatologic assessments.

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          Hemophagocytic lymphohistiocytosis: review of etiologies and management

          Hemophagocytic lymphohistiocytosis (HLH) covers a wide array of related life-threatening conditions featuring ineffective immunity characterized by an uncontrolled hyperinflammatory response. HLH is often triggered by infection. Familial forms result from genetic defects in natural killer cells and cytotoxic T-cells, typically affecting perforin and intracellular vesicles. HLH is likely under-recognized, which contributes to its high morbidity and mortality. Early recognition is crucial for any reasonable attempt at curative therapy to be made. Current treatment regimens include immunosuppression, immune modulation, chemotherapy, and biological response modification, followed by hematopoietic stem-cell transplant (bone marrow transplant). A number of recent studies have contributed to the understanding of HLH pathophysiology, leading to alternate treatment options; however, much work remains to raise awareness and improve the high morbidity and mortality of these complex conditions.
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            Treatment of adult-onset Still’s disease: a review

            Adult-onset Still’s disease (AOSD) is a rare inflammatory disorder that has been recently classified as a polygenic autoinflammatory disorder. The former classification, based on the disease course, seems to be quite dated. Indeed, there is accumulating evidence that AOSD can be divided into two distinct phenotypes based on cytokine profile, clinical presentation, and outcome, ie, a “systemic” pattern and an “articular” pattern. The first part of this review deals with the treatments that are currently available for AOSD. We then present the different strategies based on the characteristics of the disease according to clinical presentation. To do so, we focus on the two subsets of the disease. Finally, we discuss the management of life-threatening complications of AOSD, along with the therapeutic options during pregnancy.
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              Hyperferritinemia in adult onset Still's disease and the hemophagocytic syndrome.

              Increments in serum ferritin levels in adult onset Still's disease (AOSD) were reported to be higher than one could expect for a simple inflammatory state. When we analyzed the scores of 40 patients with various severe inflammatory diseases aside from AOSD, we recorded no serum ferritin values higher than 3,300 ng/ml (N less than 200 ng/ml). In 3 of 10 consecutive patients with AOSD, the ferritin levels were higher than 3,500. Among these 3 patients, one case had a ferritin value of 3,600 ng/ml and bone marrow aspirate showed a marked hyperplasia of mature appearing histiocytes, and the 2 other patients (serum ferritin levels of 65,000 ng/ml and 250,000 ng/ml) displayed the features of a hemophagocytic syndrome. In 2 patients with normal or mildly increased levels of ferritin, the bone marrow examination was normal. We suggest that very high serum ferritin levels encountered in AOSD reflect the presence of histiocytic hyperactivity that sometimes leads to a hemophagocytic syndrome.
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                Author and article information

                Contributors
                Journal
                Oxf Med Case Reports
                Oxf Med Case Reports
                omcr
                Oxford Medical Case Reports
                Oxford University Press
                2053-8855
                August 2024
                23 August 2024
                23 August 2024
                : 2024
                : 8
                : omae092
                Affiliations
                Department of Hospital Medicine, Internal Medicine Wyckoff Medical Center , 374 Stockholm Street, Brooklyn, New York, 11237, United States
                Department of Hospital Medicine, Internal Medicine Wyckoff Medical Center , 374 Stockholm Street, Brooklyn, New York, 11237, United States
                Department of Hospital Medicine, Internal Medicine Wyckoff Medical Center , 374 Stockholm Street, Brooklyn, New York, 11237, United States
                Department of Hospital Medicine, Internal Medicine Wyckoff Medical Center , 374 Stockholm Street, Brooklyn, New York, 11237, United States
                Assistant Professor New York Medical College , 40 Sunshine Cottage Rd, Valhalla, NY 10595, United States
                Author notes
                Corresponding author. Department of Hospital Medicine, Internal Medicine Wyckoff Medical Center, 374 Stockholm Street, Brooklyn, New York, 11237, United States. E-mail: zero-click@ 123456zero-click.io
                Author information
                https://orcid.org/0000-0003-1015-2393
                Article
                omae092
                10.1093/omcr/omae092
                11343824
                39184869
                ddf5b85a-1f0d-412f-a4b0-bd229516b29b
                © The Author(s) 2024. Published by Oxford University Press.

                This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( https://creativecommons.org/licenses/by/4.0/), which permits unrestricted reuse, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 13 April 2024
                : 06 June 2024
                : 25 July 2024
                Page count
                Pages: 0
                Categories
                Clinical Image
                AcademicSubjects/MED00010
                omcrep/1200
                omcrep/400
                omcrep/2300

                adult-onset still’s disease,salmon-colored rash,ferritin,artificial intelligence

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