-
Journal of Medicine and Life 2021This case report describes a rare case of progressive muscle weakness in a patient treated for eosinophilic fasciitis (EF) for many years before being diagnosed with a...
This case report describes a rare case of progressive muscle weakness in a patient treated for eosinophilic fasciitis (EF) for many years before being diagnosed with a second autoimmune disease: dermatomyositis. Our case is a report of a 65-year-old male diagnosed with eosinophilic fasciitis 7 years before being evaluated in our service at Mayo Clinic in Jacksonville, Florida, due to progressive muscle weakness despite the chronic treatment with methotrexate. Contrast-enhanced magnetic resonance imaging of the lower extremity showed enhancement throughout the thigh musculature, which led us to pursue biopsies of the fascia and muscle in order to confirm the diagnosis of EF associated with myopathy. This case illustrates the need to consider the possibility of myopathy in patients diagnosed with EF whenever muscle weakness is more prominent than expected.
Topics: Aged; Biopsy; Elbow Joint; Eosinophilia; Fascia; Fasciitis; Humans; Magnetic Resonance Imaging; Male; Methotrexate; Muscles; Muscular Atrophy; Skin
PubMed: 33767797
DOI: 10.25122/jml-2021-0015 -
BMJ Case Reports Sep 2012A 57-year-old woman presented with malaise and heaviness in her extremities. At first there were no clues of an inflammatory disease, but the patient developed slowly...
A 57-year-old woman presented with malaise and heaviness in her extremities. At first there were no clues of an inflammatory disease, but the patient developed slowly progressive oedema of her arms and legs with induration of the skin. Blood tests showed eosinophilia. Additional analysis revealed generalised lymphadenopathy. After excluding an infectious or malignant cause, the clinical diagnosis of eosinophilic fasciitis was made, this was confirmed by the results of a full thickness skin biopsy.
Topics: Anti-Inflammatory Agents; Eosinophilia; Fasciitis; Female; Forearm; Humans; Middle Aged; Prednisone; Skin
PubMed: 23035154
DOI: 10.1136/bcr.2012.6158 -
Nature Nov 2021Inflammation early in life can prime the local immune milieu of peripheral tissues, which can cause lasting changes in immunological tone that confer disease protection...
Inflammation early in life can prime the local immune milieu of peripheral tissues, which can cause lasting changes in immunological tone that confer disease protection or susceptibility. The cellular and molecular mechanisms that prompt changes in immune tone in many nonlymphoid tissues remain largely unknown. Here we find that time-limited neonatal inflammation induced by a transient reduction in neonatal regulatory T cells causes a dysregulation of subcutaneous tissue in mouse skin. This is accompanied by the selective accumulation of type 2 helper T (T2) cells within a distinct microanatomical niche. T2 cells are maintained into adulthood through interactions with a fibroblast population in skin fascia that we refer to as T2-interacting fascial fibroblasts (TIFFs), which expand in response to T2 cytokines to form subcutaneous fibrous bands. Activation of the T2-TIFF niche due to neonatal inflammation primes the skin for altered reparative responses to wounding. Furthermore, we identify fibroblasts in healthy human skin that express the TIFF transcriptional signature and detect these cells at high levels in eosinophilic fasciitis, an orphan disease characterized by inflammation and fibrosis of the skin fascia. Taken together, these data define a previously unidentified T2 cell niche in skin and functionally characterize a disease-associated fibroblast population. The results also suggest a mechanism of immunological priming whereby inflammation early in life creates networks between adaptive immune cells and stromal cells to establish an immunological set-point in tissues that is maintained throughout life.
Topics: Animals; Animals, Newborn; Cytokines; Eosinophilia; Fasciitis; Fibroblasts; Fibrosis; Health; Humans; Inflammation; Interleukin-13 Receptor alpha1 Subunit; Male; Mice; Skin; Stem Cell Niche; T-Lymphocytes, Regulatory; Th2 Cells; Wound Healing
PubMed: 34707292
DOI: 10.1038/s41586-021-04044-7 -
Cancers Sep 2023Morphea is an autoimmune fibrotic skin disease. Eosinophilic fasciitis (EF) is considered to belong to the severe spectrum of morphea. We conducted a scoping review... (Review)
Review
Morphea is an autoimmune fibrotic skin disease. Eosinophilic fasciitis (EF) is considered to belong to the severe spectrum of morphea. We conducted a scoping review assessing the risk of secondary cancer among morphea/EF patients, paraneoplastic morphea/EF and morphea/EF developing secondary to cancer therapy. The search was conducted using MEDLINE, Embase, Cochrane databases for articles published from inception to September 2022 following the Preferred Reporting Items for Systematic reviews and Meta-Analyses for Scoping Reviews (PRISMA-ScR) guidelines with no language or date restrictions. Two hundred and one studies were included. Of these, 32 studies reported on secondary cancer in morphea/EF patients, 45 on paraneoplastic morphea/EF and 125 on cancer-treatment-induced morphea/EF. While the current evidence remains limited, data suggest an increased risk of secondary cutaneous and possibly pancreatic malignancy in morphea patients, particularly the generalized subtype. There were insufficient data for EF. On the other hand, paraneoplastic morphea was anecdotal, whereas several observational studies suggested that ~10% of EF cases may be paraneoplastic, primarily in the context of hematologic malignancies. Radiotherapy-induced morphea is rare, seen in ~0.2% of treated patients and is usually localized to the treatment site, except in patients with pre-existing autoimmunity. While chemotherapy-induced cases are reported, immunotherapy morphea/EF cases are emerging and are preferentially seen with PD-1 and not CTLA-4 inhibitors. This study is limited by the type of articles included (case reports, case series and observational studies), and hence, additional research on this important topic is needed.
PubMed: 37760419
DOI: 10.3390/cancers15184450 -
Mediterranean Journal of Rheumatology Mar 2023Eosinophilic fasciitis (EF) is a rare connective-tissue disorder that is characterised by subacute onset of erythema, oedema, and induration of the skin and soft tissues... (Review)
Review
Eosinophilic fasciitis (EF) is a rare connective-tissue disorder that is characterised by subacute onset of erythema, oedema, and induration of the skin and soft tissues of the limbs and trunk. Although several triggers have been hypothesised to be associated with EF, the aetiology of eosinophilic fasciitis (EF) is still unclear, and several treatment regimens have been proposed to treat this disease. In this article, we report a case of a 72-year-old gentleman with multiple comorbidities who presented to the clinic for diffuse skin thickening present on his forearms, thighs, legs bilaterally, and over the pelvis. The patient was diagnosed with EF and failed multiple treatment regimens including prednisone, methotrexate, rituximab, but finally responded and was maintained on tocilizumab. In this article, we will review the current understanding of EF, diagnostic approach, popular treatments and review other cases of EF in which tocilizumab was used.
PubMed: 37223590
DOI: 10.31138/mjr.34.1.78 -
Pharmaceuticals (Basel, Switzerland) Feb 2023Immune checkpoint inhibitors (ICI) are the standard of care for various malignancies and have been associated with a wide spectrum of complications that are... (Review)
Review
Immune checkpoint inhibitors (ICI) are the standard of care for various malignancies and have been associated with a wide spectrum of complications that are phenotypically akin to primary autoimmune diseases. While the literature on these toxicities is growing, there is a paucity of data regarding ICI-associated scleroderma which can carry significant morbidity and limit the ability to continue effective ICI therapy. Our review aimed to analyze the current literature on ICI-associated systemic scleroderma (ICI-SSc) and key scleroderma mimics. Cases of ICI-SSc had notable differences from primary SSc, such as fewer vascular features and less seropositivity (such as scleroderma-specific antibodies and antinuclear antibodies). We found that patients with a diagnosis of SSc prior to the start of ICI can also experience flares of pre-existing disease after ICI treatment used for their cancer. Regarding scleroderma mimics, several cases of ICI-eosinophilic fasciitis have also been described with variable clinical presentations and courses. We found no cases of scleroderma mimics: ICI-scleromyxedema or ICI-scleroedema. There is a critical need for multi-institutional efforts to collaborate on developing a patient database and conducting robust, prospective research on ICI-scleroderma. This will ultimately facilitate more effective clinical evaluations and management for ICI-scleroderma.
PubMed: 37259404
DOI: 10.3390/ph16020259 -
Clinical Case Reports Dec 2020Clinical examination can be the key to pursuing a diagnosis, even in rare diseases. The "groove sign" is a typical finding in eosinophilic fasciitis and can be elicited...
Clinical examination can be the key to pursuing a diagnosis, even in rare diseases. The "groove sign" is a typical finding in eosinophilic fasciitis and can be elicited by elevation of the arm to allow for venous return with visible "grooving" of the veins due to skin thickening.
PubMed: 33363970
DOI: 10.1002/ccr3.3128 -
Dermatology Online Journal Oct 2003The case of a 76-year-old woman with eosinophilic fasciitis is presented. Reported etiologic associations and treatment options are discussed.
The case of a 76-year-old woman with eosinophilic fasciitis is presented. Reported etiologic associations and treatment options are discussed.
Topics: Aged; Eosinophilia; Fasciitis; Female; Humans; Immunosuppressive Agents
PubMed: 14594606
DOI: No ID Found -
Reumatologia 2019Eosinophilic fasciitis (EF) was described in 1974 by Shulman as a rare fibrosing connective tissue disease of unknown etiology. An undetermined trigger is thought to...
OBJECTIVES
Eosinophilic fasciitis (EF) was described in 1974 by Shulman as a rare fibrosing connective tissue disease of unknown etiology. An undetermined trigger is thought to lead to the degranulation of eosinophils that interact with fibroblasts and express fibrogenic cytokines including the transforming factor of tumor growth a and b and interleukins 1 and 6. The purpose of this study was to summarize seven cases of EF in a central hospital.
MATERIAL AND METHODS
This was a retrospective and descriptive study of a population with EF of a central hospital. All patients diagnosed with EF in a hospital unit were admitted to the study between January 1, 2005, and April 30, 2018.
RESULTS
A total of seven patients diagnosed with EF were analyzed. The median age of the population at the time of diagnosis was 56 years, and 57% of the patients were women. All patients had elevated peripheral eosinophilia and sedimentation rate, and only one patient had hypergammaglobulinemia. All patients had edema and cutaneous thickening of the limbs, 57% had constitutional symptoms, and 57% had inflammatory arthritis with joint contracture. Prednisolone (PDN) therapy was initiated in all patients, and only in two was the association of PDN with methotrexate (MTX) initially performed. In one patient triple therapy of PDN, MTX, and cyclosporine was required. At the time of this publication, only one patient maintains active disease, and tocilizumab has been initiated.
CONCLUSIONS
Recent studies show a more favorable response from the combination of PDN and MTX than from PDN alone. Considering the rarity of the disease, more long-term studies are needed regarding the etiopathogenetics, progression, recurrence of EF, and new effective therapies.
PubMed: 31130746
DOI: 10.5114/reum.2019.84813 -
World Journal of Clinical Cases Oct 2021Eosinophilic fasciitis (EF) is a rare disease characterized by inflammation of the fascia with immune system involvement. Failure to promptly diagnose and treat this...
BACKGROUND
Eosinophilic fasciitis (EF) is a rare disease characterized by inflammation of the fascia with immune system involvement. Failure to promptly diagnose and treat this disease can seriously affect the quality of life of patients. However, no clear and uniform criteria for diagnosis and treatment exist.
CASE SUMMARY
In this paper, we report two cases of EF, both of which showed symmetrical limb swelling and rigidity, increased eosinophils in the peripheral blood and bone marrow, increased red blood cell sedimentation rate, increased antinuclear antibody titer, and pathological changes in the tissues such as eosinophil and lymphocyte infiltration. Both patients were treated with hormones and cyclosporine, and showed significant improvements in their conditions.
CONCLUSION
EF is an autoimmune disease causing swelling and sclerosis of the fascia and eosinophilia. It is diagnosable by magnetic resonance imaging, positron emission tomography-computed tomography, blood routine tests, and bone marrow puncture. Glucocorticoids and immunosuppressants are effective treatments.
PubMed: 34734063
DOI: 10.12998/wjcc.v9.i29.8831