Ultrasound-guided core needle biopsy of deep fascia: A cadaveric study evaluating feasibility, accuracy and reliability.

Over the past few decades, researchers and clinicians have dedicated significant attention to fascial tissues. Current interest focuses on their anatomical and pathophysiological features. Breakthroughs in ultrasound (US) and magnetic resonance imaging (MRI) have enhanced our ability to study the dynamics and alterations of the tissue structures. However, a microscopic perspective is also essential for a comprehensive understanding of some pathologies of the fasciae. The aim of this study was to investigate, using a cadaveric study: (1) the ease of visualization of the landmarks used for the US-guided fascial core needle biopsy (CNB); (2) the consistency and accuracy of needle placement inside fascial layers using US guidance and confirmed by histological examination; (3) inter-rater reliability. We assessed the feasibility of US-guided CNB in different topographical regions of human cadavers: the thoracolumbar fascia (TLF), fascia lata (FL), and crural fascia (CF). The results, confirmed by histological examination, revealed no significant difference in needle placements between the in-plane approaches in the long and short axes for all locations and fasciae studied (long axis: 91.88%; short axis: 96.22%); p > 0.05. US-guided core needle biopsy with the in-plane approach is feasible, consistent and reliable. It could provide most or all of high-quality fascial tissue samples required for pathological examination. It could also reveal changes in fascial pathologies, capturing the exact site of pathology thanks to US guidance, in particular in patchy diseases such as eosinophilic fasciitis.

Unmasking the Veiled Intruder: A Complicated Case of Eosinophilic Fasciitis.

Eosinophilic fasciitis (EF) is a rare inflammatory disease characterized by skin and fascial thickening. Unlike systemic sclerosis, EF lacks internal organ involvement and specific autoantibodies, with peripheral eosinophilia as a hallmark feature. Patients may exhibit joint pain and contractures due to fibrosis. We present a case of a patient who presented with skin thickening involving her upper and lower extremities and was ultimately diagnosed with EF based on a skin biopsy. This case underscores the importance of recognizing the unique clinical and histological features of EF.

Joint Sparing: The Key to Unlocking Early Treatment Success in Eosinophilic Fasciitis.

Delayed diagnosis is recognized as a poor prognostic factor in eosinophilic fasciitis (EF). Elevated serum eosinophil counts, a minor criterion in the diagnostic standards, occur early in the disease course. However, signs such as the groove sign and orange-peel sign typically do not appear in the initial stages, posing challenges for early detection under the current diagnostic criteria. We report a case where the combination of "joint sparing" physical findings and elevated eosinophil counts facilitated early diagnosis and treatment. A 79-year-old woman presented with an acute onset of swelling in the upper and lower limbs. Physical examination revealed non-pitting edema with "joint sparing", and blood tests showed increased eosinophil counts. Contrast-enhanced MRI of the lower limbs showed post-contrast enhancement along the fascia, leading to a diagnosis of EF. The presence of non-pitting edema with "joint sparing" may be a valuable diagnostic indicator for EF. Furthermore, combining this with serum eosinophil counts can enable early diagnosis and treatment, potentially improving patient outcomes.

A difficult diagnosis of eosinophilic fasciitis: A case report.

Eosinophilic fasciitis can be a debilitating diagnosis and is often delayed given its similarities to other sclerotic conditions including morphea, such as bound-down indurated skin and inflammation and sclerotic thickening of tissue layers on histopathology. Delaying treatment can lead to joint contracture and residual hardness in skin which has both cosmetic and functional implications. Therefore, finding the definitive diagnosis and differentiating from other sclerotic diseases is important early in the disease course. We present a case of a 77-year-old female with a generalized rash on her back and extremities, and progressive symptoms of pain, joint contractures, and limited movement, which highlights the challenges in diagnosis and management given clinical and histological parallels between eosinophilic fasciitis and morphea.

[Eosinophilic fasciitis: From pathophysiology to therapeutics]. / Fasciite à éosinophiles : actualités physiopathologiques et nouvelles voies thérapeutiques.

Eosinophilic fasciitis (EF) is a rare connective tissue disorder characterized by painful edema and induration of the limbs and trunk, likely associated with hypereosinophilia and hypergammaglobulinemia. EF causes arthralgia and range of motion limitation, leading to significant functional impairment and poor quality of life. Since its description by Shulman in 1974, over 300 cases have been reported. We present here a review of the latest diagnostic, pathophysiological and therapeutic developments in this disease. Magnetic resonance imaging appears useful to guide diagnosis and biopsy. Diagnosis is based on a deep skin biopsy involving the fascia, which will reveal edema, sclerofibrosis of the muscular fascia and subcutaneous tissue, and an inflammatory infiltrate sometimes composed of eosinophilic polynuclear cells. EF may occur in patients treated with immune checkpoint inhibitors and the diagnosis should be raised in case of cutaneous sclerosis in these patients. The pathophysiology of the disease remains poorly understood, and its management lacks randomized, controlled, blinded trials. First-line treatment consists in oral corticosteroid therapy, sometimes combined with an immunosuppressant, mainly methotrexate. A better understanding of the pathophysiology has opened new therapeutic perspectives and clarified the role of targeted therapies in the management of EF, such as interleukin-6 inhibitors, whose efficacy has been reported in several cases.

Eosinophilic Fasciitis in a 68-Year-Old Female.

Eosinophilic fasciitis (EF) is an uncommon disorder of unknown etiology and poorly understood pathogenesis. In this report, we present a case of a 68-year-old female presented with a rapidly progressing skin tightening condition in her extremities associated with eosinophilia. Four months prior, the patient's initial complaint was skin sensitivity in the legs and forearms. Over time, this led to severe skin tightening, edema, and decreased range of motion. Clinical examination showed tightening of the skin over the anterior forearms, posterior knees, and calves without sclerodactyly or Raynaud's phenomenon. Laboratory investigations showed eosinophilia, elevated antinuclear antibody titer, and negative rheumatoid factor. This presentation raised suspicion of EF, and biopsy results showed scattered lymphocytic infiltrate involving associated fibrous tissue and perivascular lymphocytic inflammation that involved vessel walls. She was treated with low-dose steroids due to her diabetes but the stiffness continued. She was started with immunomodulators methotrexate, which showed improvement in symptoms, including softening in her arm tissues.

Juvenile eosinophilic fasciitis: a single center case series.

BACKGROUND: Eosinophilic fasciitis (EF) is a rare disease characterized by skin induration and musculoskeletal abnormalities. Diagnostic criteria for EF are based on adult populations. There is a need to expand the literature on EF in children due to limited reported cases and potential differences compared to adults. METHODS: We conducted a retrospective review of medical records for six pediatric patients diagnosed with EF at our institution between November 2011 and April 2023. Inclusion criteria required patients to be under 18 years of age at the time of diagnosis and to have confirmed diagnosis through clinical history, imaging, and histology. RESULTS: Most of our cohort were female (83%) and non-Hispanic white (50%). Age at diagnosis ranged from 4 to 16 years. Duration of symptoms before diagnosis varied from 1 to 12 months. Follow-up periods ranged from 14 to 123 months. Concurrent medical conditions included localized scleroderma, acquired thrombophilia, and juvenile idiopathic arthritis. Patients presented with progressive painful swelling, severe joint limitations, and positive prayer sign. Initial regimens involved corticosteroids and methotrexate. Hydroxychloroquine, immunoglobulin, mycophenolate mofetil, rituximab, and tocilizumab were also used depending on the patient's disease severity and course. CONCLUSIONS: Juvenile EF may manifest as swelling and progressive induration without apparent skin abnormalities. Unlike adult populations, no underlying malignancies or associations with trauma were observed in our cohort. Our cases did not exhibit systemic involvement observed in previous studies on juvenile EF. While non-specific, the prayer sign may aid in early recognition of juvenile EF and help prevent long-term disability.

The first case of SARS-CoV-2-induced eosinophilic fasciitis.

Eosinophilic fasciitis (EF), also known as Shulman syndrome, is a rare auto-immune fibrosing disorder of the fascia. Etiopathogeny of EF is still unclear. Nowadays, it is widely known that severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) may induce hyper-stimulation of the immune system. Several cases with fasciitis and rhabdomyolysis induced by coronavirus disease 2019 vaccines have been reported in the literature. Herein, we report the first case of EF possibly triggered by SARS-CoV-2 infection. A 45-year-old Tunisian woman, with no medical history, presented to our department with severe widespread muscle pain noticed one month after a SARS-CoV-2 infection. Physical examination showed an induration of the skin and subcutaneous tissue of the arms, forearms and legs with a restricted joint mobility. The level of eosinophils was 430 E/mm3 (6.1%) [1-4%]. Electromyography and creatine kinase levels were normal. Myositis-related antibodies were negative. Magnetic resonance imaging of the left arm showed high-intensity signal and thickness of the fascia without evidence of muscle or bone involvement. A muscular biopsy from the right deltoid showed thickening and inflammation of the fascia. The patient received intraveinous injections of 1000 mg of methylprednisolone for 3 days with an oral relay of 1 mg/kg per day of prednisone equivalent during 4 weeks. At one-month follow-up, a significant improvement of the skin induration and myalgia was observed, with a disappearance of the biological inflammatory syndrome. This brief report suggests a potential link between SARS-CoV-2 infection and new-onset of auto-immune fasciitis.

High-resolution musculoskeletal ultrasonography and elastography for eosinophilic fasciitis diagnosis and follow-up: a case-based review.

Eosinophilic fasciitis (EF) remains a rare condition without precise diagnostic criteria due to common symptoms with other autoimmune diseases requiring broad differential diagnosis. This paper describes the use of high-resolution musculoskeletal ultrasonography and elastography in the diagnosis and follow-up of eosinophilic fasciitis through the case of a 56-year-old male patient. In addition to laboratory data, instrumental data, and biopsy, musculoskeletal ultrasonography (US) was used both in the diagnostic process and in the follow-up period for an objective assessment of the changes in the patient's condition and response to treatment. The US showed disorganization of the myofibrils adjacent to the superficial fascia, edema, and thickening of the fascia and subcutaneous edema. In addition, the use of shear-wave elastography (SWE) demonstrated significantly reduced skin elasticity. High-frequency musculoskeletal ultrasound in combination with SWE is an effective method both for the diagnosis of EF and for the follow-up of the changes occurring after therapy. Based on the fact that it can easily differentiate the substrate of involvement, such as skin, subcutaneous tissue, or muscle fascia, ultrasound can be used to distinguish EF from other skin and muscle diseases.

Morphea, Eosinophilic Fasciitis and Cancer: A Scoping 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.

Diffuse fasciitis with eosinophilia in boys: a case-based review.

Diffuse fasciitis with eosinophilia (EF) is a rare condition classified as a part of the connective tissue disorders. The clinical presentation of this condition can be diverse, however the main symptoms include symmetrical swelling and hardening of distal parts of limbs accompanied by peripheral eosinophilia. The diagnostic criteria are not specified. In inconclusions cases Magnetic Resonance Imaging (MRI) and skin to muscle biopsy may be useful. The pathogenesis and ethiology remain unknown, but extensive physical exertion, certain infectious factors, such as Borrelia burgdorferi, or medications may serve as a trigger. EF affects equally women and men, mainly in their middle age, however the disease can occur at any age. The standard therapy contents gluccocorticosteroids. As a second-line treatment, methotrexate is usually chosen. In this article we compare world reports of EF in paediatric patients with the cases of two adolescent male patients recently hospitalized in the Department of Paediatric Rheumatology.

Diagnostic Utility of Magnetic Resonance Imaging in Eosinophilic Fasciitis: A Case Report and Review of Literature.

Eosinophilic fasciitis (EF), also known as Shulman syndrome, is a rare scleroderma-like disorder that is characterized by an acute onset of induration, swelling, erythema, and tenderness of the skin and deep fascia, often affecting all four limbs. We report a case of eosinophilic fasciitis in a 51-year-old female patient, whose diagnosis of EF was made based on the findings from clinical evaluation and magnetic resonance imaging (MRI) but without skin biopsy. She was treated with a combination therapy of prednisolone and methotrexate, and her response to therapy was assessed via clinical assessment and MRI. MRI may be a useful non-invasive diagnostic tool for not only supporting but also confirming the clinical diagnosis of EF when a skin-to-muscle biopsy is not available or cannot be performed, as well as for monitoring disease activity and response to therapy. Further prospective studies should be conducted to evaluate the precise sensitivity and specificity of MRI in diagnosing EF and also to create more structured protocols to guide the diagnosis and management of EF.

Eosinophilic fasciitis difficult to differentiate from scleroderma: A case report.

BACKGROUND: Eosinophilic fasciitis (EF) is a rare connective tissue disease that can cause swelling and sclerosis of the extremities, and special attention is needed to differentiate EF from systemic sclerosis. Misdiagnosis or omission markedly delays treatment of EF, and severe skin sclerosis in advanced stages can cause joint contracture and tendon retraction, worsening the patient's prognosis and quality of life. CASE SUMMARY: We report a case of EF in a young woman diagnosed by tissue biopsy, confirming the difficulty of differential diagnosis with scleroderma. CONCLUSION: Focusing on skin manifestations, completing tissue biopsy and radiography can help diagnose EF effectively. Clinicians should enhance their understanding of the differences between EF and scleroderma, and early diagnosis and standardized treatment can improve the prognosis of patients with EF.

Eosinophil-related diseases during treatment with glucagon-like peptide one receptor (GLP-1 RA): a case report and review of the literature.

Glucagon-like peptide one-receptor agonists (GLP-1 RA) are drugs that differ in their pharmacological composition and homology to human GLP-1 and are used most frequently for the treatment of type 2 diabetes and weight loss. There are isolated reports of eosinophilic adverse reactions associated with GLP-1 RA. We present the case of a 42-year-old female patient who, after starting weekly subcutaneous semaglutide, developed eosinophilic fasciitis with favorable clinical evolution after the discontinuation of semaglutide and the initiation of immunosuppression. A review of the eosinophilic adverse events that have been previously reported with GLP-1 RA is provided.