Background: Eosinophilic fasciitis is a rare scleroderma-like disorder characterized by inflammation and thickening of the fascia, typically presenting with skin induration, peripheral eosinophilia, and absence of systemic organ involvement. Its etiology remains unclear, although immune-mediated mechanisms and various triggers have been proposed. Case presentation: We report a case of a 49-year-old woman who developed progressive pain, stiffness, and skin induration of the upper extremities shortly after COVID-19 infection. Laboratory evaluation revealed eosinophilia, acute phase reactants and negative autoimmune serology. Magnetic resonance imaging demonstrated fascial involvement without muscle pathology. Histopathological examination confirmed eosinophilic fasciitis, showing inflammatory infiltrates and fibrosis within the fascia. The patient was treated with high-dose glucocorticoids and steroid-sparing agent – methotrexate, with subsequent clinical and laboratory improvement. At follow-up, complete resolution of symptoms was observed, reaching drug-free remission after approximately 18 months. Conclusion: This case highlights eosinophilic fasciitis as a potential post-infectious immune-mediated condition following COVID-19. Early recognition, appropriate imaging, and confirmatory biopsy are essential for timely diagnosis, appropriate treatment and favorable outcomes.

Introduction Eosinophilic fasciitis (EF) is a rare autoimmune connective tissue disease classified among scleroderma-like disorders. It is characterised by painful, woody induration of the skin of the extremities, peripheral eosinophilia, elevated erythrocyte sedimentation rate (ESR) and hypergammaglobulinemia. Case description A 28-year-old man was diagnosed with EF in August 2024 based on progressive, symmetrical induration of the skin and subcutaneous tissue of the forearms, hands, lower legs and feet, associated with restricted joint mobility. Raynaud’s phenomenon was absent. Laboratory tests revealed elevated C-reactive protein (33 mg/l), ESR (22 mm/h), peripheral eosinophilia (12.5%), and hypergammaglobulinemia (1,44 g/dl). Magnetic resonance imaging (MRI) of the left lower leg showed inflammatory oedema of the muscular fascia (Fig. 1). Histopathology revealed inflammatory infiltrates composed predominantly of CD8+ > CD4+ T lymphocytes, CD68+ macrophages and scattered eosinophils. Differential diagnosis included systemic sclerosis, other scleroderma-like syndromes, and a paraneoplastic process. Nailfold capillaroscopy was normal, antinuclear antibody test was negative, and oncological screening (computed tomography of the neck, chest, abdomen and pelvis) revealed no abnormalities. Additional investigations detected IgM antibodies in significant concentrations against Borrelia burgdorferi, and doxycycline therapy was administered for 28 days. Initial treatment with oral glucocorticosteroids (GCs; prednisone equivalent 0.5 mg/kg/day) was ineffective. Subsequently, i.v. high-dose methylprednisolone pulse therapy (500 mg/day for 5 days) was administered, and methotrexate was introduced up to a target dose of 25 mg/week. Due to persistent disease activity and GC dependence, after reviewing the literature, treatment with the interleukin-6 (IL-6) inhibitor tocilizumab (162 mg s.c. once weekly) was initiated in January 2025. This resulted in improved joint mobility, stabilisation of skin induration, normalisation of eosinophil count and inflammatory markers, and allowed tapering of methylprednisolone to 6 mg/day. Follow-up MRI in January 2026 demonstrated near-complete resolution of fascial inflammatory changes (Fig. 2). Given the favourable response to anti-IL-6 therapy, treatment was continued. Conclusions This case highlights the efficacy of tocilizumab in refractory and GC-dependent EF, leading to clinical and laboratory remission as well as significant improvement on imaging. Interleukin-6 inhibitors may represent a valuable therapeutic option in severe forms of eosinophilic fasciitis.

Eosinophilic fasciitis is a rare connective tissue disorder characterized by fascial inflammation and eosinophil infiltration. Thromboembolic complications in eosinophilic fasciitis are uncommon and poorly understood. We report a case that highlights the limitations of conventional anticoagulation in preventing eosinophil-mediated thrombosis. A 60-year-old Chinese man presented with a 10-day history of progressive bilateral lower extremity swelling and pain. Laboratory investigations revealed severe hypereosinophilia and imaging revealed left lower-extremity deep vein thrombosis complicated by acute compartment syndrome. Despite therapeutic enoxaparin anticoagulation with documented hypocoagulable state on thromboelastography, the patient developed bilateral lacunar infarctions on day 7. Bone marrow aspiration excluded hematological malignancies, and fascial biopsy confirmed eosinophilic fasciitis. Following glucocorticoid therapy initiation, eosinophil levels normalized with complete resolution of symptoms. No recurrence of thrombotic events was observed during one-year follow-up. This case highlights that eosinophilic fasciitis-associated hypereosinophilia can induce a prothrombotic state resistant to conventional anticoagulation, likely through eosinophil granule protein-mediated mechanisms. Early recognition and prompt glucocorticoid therapy, rather than anticoagulation alone, may be critical for preventing life-threatening thromboembolic complications in eosinophilic fasciitis. This report adds to the limited literature on thrombotic manifestations of eosinophilic fasciitis and underscores the need for heightened clinical vigilance and a shift in therapeutic paradigm toward early immunosuppression.

Eosinophilic fasciitis (EF), or Shulman syndrome, first described in 1974, is a rare fibrosing disorder characterized by painful, symmetric swelling and progressive woody induration of skin and subcutaneous tissues. Although the pathogenesis remains unclear, EF is considered immune-mediated, often triggered by physical exertion, infections, or medications. Pediatric EF, a particularly uncommon subset, can exhibit distinct clinical features, including pronounced extracutaneous manifestations, unpredictable disease progression, and variable therapeutic responses. Clinical presentations range from rapidly advancing fibrosis leading to joint contractures to fluctuating inflammatory episodes. Diagnosis is challenging because of the absence of universal criteria, although peripheral eosinophilia, elevated inflammatory markers, and imaging findings support clinical suspicion. Definitive diagnosis depends on deep skin and fascial biopsy, revealing eosinophil-rich lymphoplasmacytic infiltrates and fibrosis. Management primarily relies on systemic corticosteroids, supplemented by steroid-sparing immunosuppressive medications in refractory cases. Early diagnosis and treatment are critical because untreated EF can cause irreversible fibrosis and significant functional impairment. The disease's rarity, heterogeneous presentations, and unclear etiology further complicate clinical management. Recent insights suggest EF may involve intricate interactions among environmental triggers, immune dysregulation, and fibrotic remodeling. This review aims to provide an updated overview of pediatric EF, highlighting current knowledge on clinical manifestations, diagnosis, differential diagnosis, therapeutic approaches, and outcomes, supported by an illustrative case, with emphasis on areas needing further research.

Eosinophilic Fasciitis and Morphea Share Gene Signatures of Inflammatory Cell Death, Self-DNA Recognition, and Enhanced Jak/Signal Transducer and Activator of Transcription Signaling.

Necrotizing fasciitis (NF) is known to be apotentially life-threatening bacterial infection of the deep fascias showing atendency to rapid spread. For certain localizations (e.g., the scrotum or labia) of this disease, specific entities have been defined (e.g., Fournier's gangrene). Cellulitis (or phlegmon) represents the most important differential diagnosis of NF. Moreover, septic soft tissue infections with fascial involvement together with eosinophilic and paraneoplastic fasciitis can be diagnostically challenging. The large group of autoimmunologically mediated inflammatory systemic disorders (such as lupus myofasciitis) at least shares some radiological similarities, although both its bilateral and partially symmetrical involvement pattern as well as the overall clinical constellation would argue against it.

ABSTRACTEosinophilic fasciitis (EF) is a rare condition with an unknown cause. This case study showed that a 40‐year‐old man with EF did not respond to standard treatments but improved after receiving rituximab and intravenous immunoglobulin (IVIG). Further studies are needed to confirm rituximab's effectiveness and long‐term safety for EF.

Idiopathic inflammatory myopathies (IIM) constitute a heterogeneous group of rare autoimmune, chronic inflammatory muscle disorders, resulting in both musculoskeletal and systemic manifestations. They are the leading cause of potentially treatable myositis in both pediatric and adult populations. Precise diagnosis is imperative, as treatment options differ for each disease, directly influencing the patient's therapeutic response and prognosis. The integration of pathological, clinical, serological, and imaging data is vital because of the nonspecific or nearly normal histopathological findings observed in certain cases. Magnetic resonance imaging (MRI) is the preferred imaging modality for IIM assessment, capable of revealing disease activity and distribution patterns, and assisting in the selection of biopsy sites. Advanced techniques such as diffusion-weighted imaging and whole-body MRI have enhanced MRI performance in evaluating inflammatory activity and disease progression. This review examines the clinical and MRI characteristics of the subtypes of IIM, and addresses other important differentials, namely eosinophilic fasciitis and vasculitis with muscle involvement, emphasizing recent advancements in imaging techniques and the significance of a comprehensive approach in myositis evaluation. KEY POINTS: Question IIM and other myositis pose significant diagnostic challenges due to overlapping characteristics. Can advanced imaging modalities improve diagnostic accuracy, monitoring, and treatment decisions? Findings MRI, with emphasis on whole-body MRI, is highly effective in evaluating myopathies, facilitating disease activity detection, guiding biopsies, and differentiating subtypes through distinct imaging patterns. Clinical relevance Advanced imaging techniques contribute to early and precise diagnosis of IIM, optimize therapeutic strategies, and enhance disease monitoring. Whole-body MRI is instrumental in identifying systemic involvement, enabling timely intervention, and improving patient outcomes.

Eosinophilic fasciitis, also known as Shulman’s disease, is a rare scleroderma-like fibrosing disorder characterized by inflammation and thickening of the fascia, often associated with peripheral eosinophilia. Because of its rarity and heterogeneous clinical presentation, EF is frequently misdiagnosed, particularly as systemic sclerosis or other connective tissue diseases. We report the case of a 28-year-old woman with no significant past medical history who presented with a two-week history of painful swelling and progressive induration of the upper and lower limbs, associated with morning stiffness and functional impairment. There was no Raynaud’s phenomenon, no digital ulcers and no visceral involvement. Laboratory investigations revealed marked peripheral eosinophilia at 1,100/mm³, autoimmune serology, including antinuclear and extractable nuclear antigen antibodies, was negative. Magnetic resonance imaging of the affected limbs demonstrated diffuse thickening and enhancement of the superficial and deep fasciae. A full-thickness skin-to-muscle biopsy confirmed the diagnosis by showing dense inflammatory infiltrates rich in eosinophils within the fascia, with associated collagen thickening and fibrosis. The patient was treated with three consecutive daily pulses of intravenous methylprednisolone, then switched to oral prednisone at 1 mg/kg/day with gradual tapering. Clinical response was rapid, with significant improvement in pain, regression of edema and induration, and normalization of eosinophil count and inflammatory markers. This case highlights the importance of considering eosinophilic fasciitis in patients presenting with limb edema and skin induration without Raynaud’s phenomenon, especially in the presence of unexplained eosinophilia. Early recognition and prompt initiation of systemic corticosteroid therapy are crucial to prevent irreversible fibrosis and functional sequelae.

This article reports the diagnosis and treatment process of a 52-year-old female patient who was finally diagnosed with POEMS syndrome. Her main clinical manifestations included Raynaud phenomenon of both hands, skin pigmentation, swelling of both hands and feet, and numbness of both feet. The patient was admitted to the Department of Rheumatology and Immunology, Peking University People's Hospital on April 8, 2024, due to "purplish red skin on the neck and chest for 1.5 years, swelling of both hands for 1 year, and numbness of both feet for 8 months". One and a half years ago, she was diagnosed with systemic sclerosis (SSc) in another hospital, based on evidence from a series of clinical manifestations. Her main symptoms were Raynaud phenomenon of both hands, skin pigmentation, swelling of both hands and feet, and numbness of both feet, which met the 2013 classification criteria for SSc by the American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR). During the disease course, the patient received regular treatment with glucocorticoids and disease-modifying antirheumatic drugs (DMARDs), but her condition still progressed continuously. Eight months ago, she developed new-onset numbness in both her feet accompanied by pinprick-like pain, indicating the complexity of the condition and the need for further investigation of the etiology. Differential diagnosis should consider possibilities, such as mixed connective tissue disease, eosinophilic fasciitis, and malignant tumors. Auxiliary examinations showed that the patient's serum antinuclear antibody, anti-topoisomerase Ⅰ(Scl-70) antibody, anti-U1 ribonucleoprotein (RNP) antibody, and antineutrophil cytoplasmic antibody were all negative. Imaging examinations revealed no pulmonary arterial hypertension or pulmonary interstitial fibrosis. In addition, the patient had multiple endocrine abnormalities and responded poorly to treatment with glucocorticoids and DMARDs, suggesting the need to be alert to the possibility of lymphoproliferative diseases. Further examinations including vascular endothelial growth factor detection, whole-body bone scan, and bone marrow aspiration and biopsy were performed, and the final diagnosis of POEMS syndrome was confirmed. The patient was then transferred to the Department of Hemato-logy and received treatment with the pomalidomide combined with dexamethasone regimen, and her clinical symptoms gradually relieved. This case suggests that POEMS syndrome is similar to rheumatological and immunological diseases such as SSc in terms of clinical manifestations. Clinicians should be more vigilant during diagnosis and treatment, and pay attention to differentiation, so as to reduce missed diagnoses and misdiagnoses, thereby formulating more accurate and effective treatment plans for patients.

A 55-year-old male patient presented with generalized myofascial complaints. The clinical and laboratory investigations were unremarkable apart from taut skin of the lower extremities and eosinophilia of the blood. Based on the enrichment of the fascias seen in magnetic resonance imaging (MRI) and the detection of inflammatory epifascial infiltrates in the muscle biopsy, the diagnosis of eosinophilic fasciitis was made. Treatment with glucocorticoids, methotrexate and tocilizumab led to acomplete clinical and morphological regression of the fasciitis.

Background. Eosinophilic fasciitis (EF) is a rare inflammatory condition classified among scleroderma-like syndromes. Corticosteroids represent the first-line treatment, followed by conventional immunosuppressants. In refractory cases, other agents have been used, including rituximab (RTX), although it is not currently included in EF guidelines (1). Our study aimed to evaluate the efficacy and safety of RTX by describing three cases from our centre and conducting a systematic review of the literature. Methods. We included patients with a clinical and histological diagnosis of EF1 who were treated with RTX at our centre between 2010 and 2025. Demographic, clinical, laboratory, and instrumental data, as well as previous and concomitant therapies, were collected. Disease severity was assessed according to the Jinnin et al. score. A systematic review of the literature (01/2010–05/2025) was conducted in accordance with PRISMA guidelines using the Medline (PubMed) database, including studies published in English on adult patients with EF (population) treated with RTX (exposure), with the aim of evaluating efficacy and safety (outcomes). Results. Three patients with EF who were treated with RTX were identified. Demographic and clinical features are summarized in Table 1. All patients had typical skin involvement confirmed by histology. Physical triggers, infections, drugs, autoimmune diseases, oncological and hematological diseases were excluded. At the time of diagnosis, all patients presented with peripheral eosinophilia and were initially treated with corticosteroids and methotrexate. RTX was added due to progressive skin involvement and a worsening disease severity score. A rapid clinical improvement was observed in all cases, with a reduction in skin thickening, extent of involvement, and disease severity score. The mean daily corticosteroid dose at the last follow-up was 71% lower compared to the pre-RTX dose. In terms of safety, two non-serious adverse events were reported: hypogammaglobulinemia and an upper respiratory tract infection in one patient. A review of the literature identified ten additional cases of EF treated with RTX. Demographic, clinical, and treatment characteristics are reported in Table 2. RTX therapy was clinically effective in eight out of ten cases. Conclusions. Rituximab may represent an effective and safe therapeutic option for patients with EF who are refractory to corticosteroids and conventional DMARDs. Both our case series and the literature data support the use of RTX in EF unrelated to hematologic conditions, highlighting its potential as a steroid-sparing agent with a favourable safety profile.

Background. Stiff skin syndrome is an extremely rare genetically determinated connective tissue disorder characterized by progressive skin induration and requiring complex differential diagnosis with autoimmune pathology. The lack of information about the disease course in children, the limited availability of treatment options, and the uncertain prognosis determine the high clinical significance of each carefully documented case. Purpose – to present a unique clinical case of stiff skin syndrome in a child in Ukraine with a detailed analysis of clinical manifestations, instrumental and laboratory investigations, differential diagnosis, and the effectiveness of the administered treatment. Materials and Methods. A retrospective analysis of a 7-year-old child with progressive skin induration and limited mobility was performed. Diagnosis was established using clinical and anamnestic data, laboratory findings, and the results of instrumental, histological, and immunohistochemical examinations. Results. The patient showed typical manifestations of stiff skin syndrome including diffuse skin induration, limited movements, contracture formation, limb asymmetry and gait disturbance without signs of systemic inflammation. MRI confirmed that the lesions were confined to the skin, without involvement of the fascia or internal organs. Histologically, thickening and structural disorganization of collagen fibers, hyperkeratosis, and fibrosis were identified. Differential diagnosis was performed to distinguish the condition from systemic scleroderma, eosinophilic fasciitis, and other sclerosing dermopathies. Anti- inflammatory and cytostatic therapy were ineffective, while rehabilitation measures contributed to partial improvement in the patientʼs functional status. Conclusions. The presented case highlights the complexity of diagnosing stiff skin syndrome and the necessity of excluding other scleroderma-like diseases. Given the limited effectiveness of pharmacological treatment, physical rehabilitation is a key component in the management of such patients.

Rationale:Eosinophilic fasciitis (EF) is a rare fibrosing disorder with an undetermined etiology and an incompletely elucidated pathogenesis. Although eosinophilia is frequently observed during the acute phase and serves as a diagnostically suggestive feature, its absence does not exclude active disease. Notably, the incidence of eosinophilia shows a progressive decline in chronic cases, correlating with the disease duration and fibrotic progression.Patient concerns:A 42-year-old male manual laborer presented with a 12-month history of progressive symmetric induration and hyperpigmentation affecting all 4 limbs.Diagnoses:Blood tests revealed eosinophilia (1.52 × 10⁹/L), and magnetic resonance imaging demonstrated characteristic hyperintensity of the fascial layers on T2-weighted imaging.Interventions:The patient was initially treated with low-dose corticosteroids combined with methotrexate.Outcomes:Laboratory monitoring confirmed complete normalization of the previously elevated peripheral eosinophil count, indicating a favorable treatment response.Lessons:EF is a rare connective tissue disorder that frequently poses diagnostic challenges, often leading to missed or incorrect diagnoses in clinical practice. However, contemporary diagnostic approaches enable the accurate identification of suspected EF cases through comprehensive evaluation of characteristic clinical manifestations, laboratory findings, and imaging features, even in the absence of confirmatory biopsy.

Eosinophilic fasciitis.

Patient: Male, 78-year-oldFinal Diagnosis: Eosinophilic fasciitisSymptoms: Pain and diffuse hardening of the skin in his extremities and backClinical Procedure: —Specialty: RheumatologyObjective: Rare diseaseBackgroundImmune checkpoint inhibitors (ICI) like pembrolizumab are increasingly used in cancer treatment and have become the standard of care for certain types of malignancies. Expanded use of these medications has led to more frequent recognition of immune-related adverse events (irAEs), including those presenting with sclerosing skin conditions such as eosinophilic fasciitis (EF). This case report describes the clinical presentation and management of a 78-year-old man with a history of bladder cancer who developed eosinophilic fasciitis after 6 months of treatment with an ICI.Case ReportFollowing 6 months of treatment with pembrolizumab, the patient developed capillary-leakage syndrome (CLS), which was treated with intravenous immunoglobulin (IVIG) and steroids. Several months after the resolution of his CLS, he developed painful hardening of the skin in his extremities and back, with notable induration of the skin and restricted range of motion across his elbows on physical examination. Laboratory workup revealed elevated inflammatory markers and eosinophilia. Skin biopsy revealed fibrosis of subcutaneous tissue and deep fascia, with multifocal collections of lymphocytes and plasma cells, consistent with eosinophilic fasciitis. Pembrolizumab was discontinued, and he received treatment with prednisone, mycophenolate mofetil, hydroxychloroquine, and benralizumab, with some improvement in his skin lesions.ConclusionsThis case report supports the need to have a high index of suspicion and investigate for eosinophilic fasciitis in cancer patients treated with anti-PD-1 medications who present with symmetric, painful hardening of the skin.

Fibrotic skin diseases are rare, chronic, and often debilitating conditions characterized by excessive extracellular matrix deposition, leading to tissue scarring and functional impairment. Despite their severity, diseases—such as lichen sclerosus et atrophicus (LSA), frontal fibrosing alopecia (FFA), radiation-induced skin fibrosis (RISF), eosinophilic fasciitis (EF), pansclerotic disabling morphea (PDM), and linear circumscript sclerodermia (LCS)—lack approved therapies and are underrepresented in clinical research. This phase 2b multicenter basket trial proposes a novel approach to evaluate a common antifibrotic therapy across these diverse but pathophysiologically related conditions. The trial employs a two-stage Simon design to address the statistical challenges posed by small patient populations, allowing the inclusion of ultra-rare diseases while maintaining analytical rigor. LSA and FFA serve as primary study groups due to higher prevalence, while EF, RISF, PDM, and LCS are included as exploratory arms. The study aims to assess the efficacy, safety, and tolerability of the selected therapy, while also providing mechanistic insights into fibrosis through molecular analyses. The primary endpoint is a ≥ 1-point improvement in the Investigator Global Assessment (IGA) at 24 weeks. Secondary endpoints at 52 weeks encompass quality of life (Dermatological Life Quality Index (DLQI), EuroQol Group Quality of Life Questionnaire (EuroQol five dimensions (EQ-5D))), symptom relief (itch and pain Numeric Rating Scale (NRS)), and disease-specific clinical scores. The trial excludes a placebo arm due to ethical considerations in progressive, untreated diseases but allows rescue therapies for disease progression. This design not only facilitates access to treatment for underserved populations but also leverages shared clinical and molecular features to enhance statistical power. By integrating disease-specific and global outcome measures, the study aims to generate robust evidence for repurposing existing therapies. If successful, this trial could serve as a model for future research in rare fibrotic diseases, accelerating drug development and improving patient outcomes.

Eosinophilic fasciitis associated with Sjögren’s disease: a rare case

Background: Morphea is a rare sclerosing disorder characterized by the formation of erythematous to violaceous lesions and sclerotic plaques on the skin that can extend to the reticular dermis and subcutaneous tissue. This condition may be associated with pain, pruritus, and limitations in range of motion, causing significant disruption in patients’ quality of life. Similar sclerosing disorders, such as eosinophilic fasciitis and systemic sclerosis, have been associated with hematologic diseases like aplastic anemia (AA). However, few reports of morphea in association with AA have explored this relationship. Case presentation: A 29-year-old woman with a history of AA presented with pruritic, indurated hyperpigmented plaques on her lower extremities. Her past medical history included congenital aortic stenosis and focal epilepsy. A skin biopsy was consistent with morphea. A concurrent bone marrow biopsy confirmed persistent hypocellularity consistent with AA. Conclusion: This case highlights a potential immunologic link between morphea and AA given that both diseases involve dysregulated T cell-mediated immune responses. We explore a potential immunologic connection between the two conditions, including the role of chemokines, such as CXCL10, in their pathogenesis. Additionally, we discuss important considerations for physicians when evaluating patients with morphea.

Eosinophilic fasciitis (EF) is a rare fibrosing disorder caused by an autoimmune response to an unknown trigger. Many possible triggers have been suggested including strenuous exercise, drug or chemical exposure, and preceding infection. We present a case of a female patient, age 69 years, who developed EF following SARS-CoV-2 infection. There have been several advances in the diagnosis and management of EF since it was first described 50 years ago. EF is a mimic of scleroderma, but key clinical features can be used to differentiate between the two diagnoses. Laboratory abnormalities include eosinophilia, elevated inflammatory markers, and hypergammaglobulinemia. A full thickness biopsy of the skin including muscle and fascia is recommended to confirm the diagnosis. Imaging modalities such as ultrasound and magnetic resonance imaging have been increasingly used in the diagnosis and follow-up of EF. Corticosteroids remain the first line in treatment of EF. Combination of steroids and methotrexate have shown the best possible outcome. Early diagnosis is important for better treatment response.