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Eosinophilic fasciitis (EF, also known as Shulman syndrome) is an uncommon connective tissue disease characterized by inflammatory thickening of the fasciae as well as swelling and hardening of the skin. It mostly affects the lower extremities. Swollen and indurated skin, together with the groove sign, are typical clinical signs. So far, biopsy evidence of inflammation and thickening of the fascia has been the gold standard for diagnosis. Magnetic resonance imaging (MRI) is mentioned in the literature as an alternative method for confirming the diagnosis. We present a case of asymmetric EF in a 54-year-old German male. He came with painful induration of the right forearm, with a characteristic groove sign and limitation of motion of the right hand. The blood count revealed eosinophilia with 0.57 G/l or 9.6% (normal: 0.05-0.5 G/l and 0.5-5.5%), ANA and ENA were negative. The diagnosis was confirmed histologically and we were able to detect a thickened fascia in MRI and ultrasound imaging. The EF also appeared in the left lateral malleolus during the course of the illness. Treatment was carried out with prednisolone and methotrexate., (© 2022. The Author(s).)

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., (© 2024. The Author(s).)

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., (© Japan College of Rheumatology 2023. Published by Oxford University Press.)

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., (© 2023. The Author(s).)

Purpose: Below knee amputation (BKA) is a common surgical procedure for diabetic foot ulcers and necrotizing lower limb fasciitis patients. However, it is a painful procedure and inadequate postoperative analgesia impedes rehabilitation and prolongs hospitalization. An ideal pain management regimen should provide superior analgesia while minimizing opioid consumption and improving rehabilitation., Methods: We retrospectively reviewed medical charts of 218 patients who underwent BKA for diabetic foot ulcer or necrotizing lower limb fasciitis at a single center between January 2017 and September 2020. Two groups were analyzed: patients who received dual nerve block (DNB) before surgery (Group I; n = 104), and patients who did not (Group II; n = 93). By the exclusion criteria, 21 patients were excluded. The femoral and sciatic nerves were each blocked separately under ultrasound guidance. This procedure was performed immediately before the operation., Results: Group I patients' subjective pain scores were significantly lower than that of Group II at 6, 12, and 24 h after BKA (P < 0.05). Group I's morphine milligram equivalent (MME) was significantly lower than those of Group II at 72 h after BKA (P < 0.05). Moreover, the rate of postoperative nausea and vomiting (PONV) and delirium was significantly lower in Group I patients than that in Group II patients., Conclusion: Ultrasound-guided lower extremity nerve block surgery is excellent for early postoperative pain control, could be used as an accurate and effective pain control method, and can reduce the side effects of opioid consumption after BKA., (© 2023. BioMed Central Ltd., part of Springer Nature.)

Eosinophilic fasciitis (EF), defined as diffuse fasciitis with eosinophilia by Shulman in 1974, is a disease with unknown etiology and whose pathogenesis is still being researched. The diagnosis is based on the clinical aspects (skin induration with an "orange peel" appearance), the lab results (eosinophilia, increased inflammatory markers), the skin biopsy with the pathognomonic histopathological result, as well as the typical MRI changes. The treatment includes glucocorticoids and immunosuppressive drugs. Due to severe refractory cases, the treatment remains a challenge. EF is still a disease with potential for further research.

A 58-year-old man presented with painful edema of the extremities, and a diagnosis of eosinophilic fasciitis (EF) was confirmed. He also met the criteria for hypereosinophilic syndrome (HES), but there were no findings suggestive of malignancies or hematologic neoplasms despite a close examination. He was started on steroid therapy but subsequently developed severe liver dysfunction, hemophagocytic lymphohistiocytosis, hepatosplenomegaly, and renal involvement. The diagnosis of peripheral T-cell lymphoma, not otherwise specified was finally established by a bone marrow reexamination and liver biopsy. In cases of eosinophilia, EF, and/or HES, it is important to suspect an intrinsic abnormality, including potential T-cell lymphoma.

Background: Eosinophilic fasciitis (EF) is an extremely rare disease with polymorphic presentation and prognosis., Objective: To further investigate EF features., Methods: We performed a retrospective multicentre study of EF patients from 2013 to 2019, clustered patients using multivariate correspondence analysis, and sought prognosis factors., Results: One hundred twenty-eight patients were included. Sixty-nine (50%) patients had skin sclerosis, and eosinophil count was increased in 71 (55%) patients. Multivariate correspondence analysis identified 3 clusters: a "mild," a "late-onset and hypereosinophilic," and a "fibrotic" cluster. Of 109 patients who followed up for more than 1 year, 49 (45%) presented a relapse, and 48 (44%) had sequelae. Multivariate analysis revealed that eosinophilia (hazard ratio = 1.56; P = .02) and fibrosis (hazard ratio = 4.02; P = .002) were predictive factors of relapse, whereas edema (odds ratio = 0.31; P = .03), relapse (odds ratio = 3.00; P = .04) and fibrosis (odds ratio = 1) were predictive factors of sequelae. Following relapse, treatment modifications consisted of an increase in glucocorticoids in 40 (82%) patients and the addition of methotrexate in 31 (63%) patients. These modifications led to clinical improvement and glucocorticoid withdrawal in 37 (76%) and 22 (45%) patients., Limitations: Retrospective study., Conclusion: EF patients can be divided into 3 homogenous clusters, which, along with fibrosis and eosinophilia, are prognosis factors of relapse and sequelae., Competing Interests: Conflicts of interest None disclosed., (Copyright © 2022 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.)

Palmar fasciitis and polyarthritis syndrome (PFPAS) is a rare paraneoplastic rheumatic disease with characteristic features. We herein report a 77-year-old man with lung adenocarcinoma and contralateral pulmonary metastasis receiving chemotherapy who presented with progressive symmetrical flexion contractures associated with palmar fascial thickening and arthritis of both hands and shoulders. He was diagnosed with PFPAS as paraneoplastic manifestations. Salazosulfapyridine was not effective, but 15 mg/day of oral prednisolone improved his symptoms. Physicians should consider PFPAS and rule out malignancy in patients with arthritis in the extremities and flexion contractures associated with palmar fascial thickening.

Introduction: Eosinophilic fasciitis (EF) is a rare autoimmune disease causing progressive induration of dermal, hypodermal, and muscularis fascia. The exact pathogenesis is yet to be fully understood, and a validated therapy protocol still lacks. We here aimed to realize a clinical-functional characterization of these patients., Materials and Methods: A total of eight patients (five males, 45 years average) were treated with adjuvant high-dose UVA-1 phototherapy (90 J/cm), after having received the standard systemic immunosuppressive protocol (oral methylprednisolone switched to methotrexate). Body lesion mapping, Localized Scleroderma Assessment Tool (LoSCAT), Dermatology Life Quality Index (DLQI), High-Resolution Ultrasound (HRUS) (13-17MHz), and ultra HRUS (55-70 MHz) were performed at each examination time taking specific anatomical points. Gene expression analysis at a molecular level and in vitro UVA-1 irradiation was realized on lesional fibroblasts primary cultures., Results: The LoSCAT and the DLQI showed to decrease significantly starting from the last UVA-1 session. A significant reduction in muscularis fascia thickness (-50% on average) was estimated starting from 3 months after the last UVA-1 session and maintained up to 12 months follow-up. Tissues was detected by HRUS. The UVA-1 in vitro irradiation of lesional skin sites cells appeared not to affect their viability. Molecular genes analysis revealed a significant reduction of IL-1ß and of TGF-ß genes after phototherapy, while MMPs 1,2,9 gene expression was enhanced., Comment: These preliminary in vivo and in vitro findings suggest that UVA-1 phototherapy is a safe and useful adjuvant therapy able to elicit anti-inflammatory effects and stimulate tissue matrix digestion and remodeling at lesional sites., (© 2021 The Authors. International Journal of Dermatology published by Wiley Periodicals LLC on behalf of the International Society of Dermatology.)

Eosinophilic fasciitis (EF) is an uncommon autoimmune connective tissue disorder characterized by edema, erythema, and subsequent induration of the extremities. It is commonly treated with corticosteroids but there is no treatment ladder for immunosuppressants or steroid-sparing agents. We report two EF cases treated effectively with mycophenolate mofetil (MMF) or mycophenolic acid (MPA) and present a literature review. We performed a MEDLINE search using the keywords 'eosinophilic fasciitis', 'Shulman syndrome', 'mycophenolic acid', or 'mofetil mycophenolate', and found 8 articles with 27 cases in which MMF or MPA was used. Twenty-nine cases were reviewed (2 reported herein and 27 from the literature search); all patients received a combination of systemic corticosteroids and MMF. MMF/MPA were given as a steroid-sparing agent in 27 (93.1%), in 1 (3.4%) as adjunctive therapy with other immunosuppressants, and in one, as monotherapy 1 (3.4%). Nineteen had a complete response, 6, a partial response, and 2 were unresponsive to diverse immunomodulators; in 2 cases, the outcome was not reported. MMF and MPA show promising therapeutic results and could be a treatment option to reduce corticosteroid related side effects.

Competing Interests: The authors declare no conflict of interest.

Key Message: RTX could be an effective and safe alternative treatment for refractory EF. Rituximab (RTX) is a successful therapeutic option for various autoimmune diseases. Our aim is to report our experience with RTX in eosinophilic fasciitis (EF) and review published data on its efficacy for the treatment of EF. We reviewed the medical charts of all patients with a diagnosis of EF treated with RTX from 2008 to 2020 in the Department of Rheumatology and Clinical Immunology in the University Hospital of Heraklion, Crete, Greece. We also reviewed the English literature for cases of EF treated with RTX. Demographics, clinical manifestations, laboratory findings, prior treatments, response to RTX, cumulative RTX dose, duration of treatment and follow-up are reported. We report three cases of EF refractory to conventional DMARDs (cDMARDs) that responded to RTX. Furthermore, literature review revealed five cases. In our case series in all patients, RTX was the first biologic. RTX could be effective in cases of (EF) refractory to standard immunosuppressive treatment., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Familial Mediterranean fever (FMF) is an autoinflammatory disease characterized by recurrent febrile attacks and serositis. The diagnosis of FMF has been based on clinical criteria, including frequent symptoms and good response to the treatment with colchicine. Some patients with FMF show skin or muscle manifestations, which may be confused with other cutaneous or muscle disorders. Here we report a female in her 40s with periodic fever, migratory myalgia, dermatomyositis-like dermatitis, arthralgia, pharyngalgia, and lymphadenopathy. The initial clinical differential diagnosis included dermatomyositis, malignant lymphoma, and adult-onset Still's disease. However, the following examinations could not explain her pathological condition with such diseases. In particular, findings from muscle and fascial biopsy demonstrated severe inflammatory cell infiltrate in the fascia, suggesting fasciitis as a possible cause of migratory myalgia. We examined the possibility of autoinflammatory diseases by genetic testing. Accordingly, she was found to have novel compound heterozygous mutations (L110P, E148Q, and P369S) in the MEFV gene. Given her genetic mutations and favorable response to colchicine, she was finally diagnosed as a variant of FMF with myalgia and previously unprecedented skin eruptions., (© 2021 Japanese Dermatological Association.)

Eosinophilic fasciitis (EF) and generalized morphea (GM) are rare and difficult-to-treat sclerosing skin diseases which may occur in association with hematologic disorders. We present a 66-year-old man with EF and associated Waldenström macroglobulinemia who received combination therapy with rituximab (375mg/m2 every other week, gradually extended to every eight weeks), prednisolone (1.25-30mg/d), and methotrexate (7.5-15mg/w). Three months after rituximab initiation, his skin condition improved steadily accompanied by a significant improvement in joint mobility with only mild and transitory flares (observation period: 59 months under treatment with rituximab). To date, there are five case reports on rituximab treatment of EF/GM with an association to hypergammaglobulinemia in three of those cases. Therapy effected significant improvement in four patients. Our case adds to the hitherto limited evidence that rituximab may be a promising therapeutic strategy for EF/GM in association with hypergammaglobulinemia.

Eosinophilic fasciitis (EF) is characterized by symmetrical thickening of subcutaneous muscular fascia, causing skin induration with wrinkles and prominent hair follicles: the classic peau d'orange. Eosinophilia is a characteristic-albeit not universal-finding. We present the case of a 43-year-old pregnant woman diagnosed with EF during pregnancy who had extensive cutaneous involvement and severe functional repercussions, including worsening of lung function and intrauterine growth restriction as a possible complication. Treatment with prednisone was initiated during gestation and it was necessary to increase the dose. After delivery, methotrexate treatment was initiated and the corticosteroid dose progressively decreased, with progressive worsening in the torso and abdomen and secondary dyspnea due to thoracic pressure. Treatment with infliximab was then initiated, with favorable progress, though residual ankle and tarsal joint stiffness and significant muscular atrophy in the limbs continued. The triggering factor of EF was not identified. In a systematic search of the medical literature, three cases of EF in pregnant woman without clear triggers were found. Interestingly, all three cases progressed favorably with steroid treatment. Apart from this case, there are only seven published cases of infliximab use in the literature, all with moderate or complete response. Infliximab could be an option for corticosteroid-dependent EF with no response to other options.

Nodular fasciitis (NF) is a subcutaneous, nodular, pseudo-sarcomatous, fibroblastic proliferation. It is rarely reported in the periorbital region and the management approach is variable.Presented is an eight-year-old female with a three month history of a periorbital mass. Incisional biopsy histologically confirmed nodular fasciitis with a unique gene translocation. The lesion was treated primarily with one intra-lesional injection of triamcinolone acetonide. Four months post-injection, the lesion resolved completely. No recurrence was seen at 12-months follow-up post-injection. No side effects were noted.To our knowledge. this is the first reported use of intra-lesional triamcinolone acetonide as a first-line treatment in periorbital NF. We found this to be a safe and effective treatment, which can obviate the need for surgical excision in a cosmetically sensitive region.

Eosinophilic fasciitis (EF) is a rare connective tissue disease that causes inflammation and fibrosis of the fascia, inducing pain and motor dysfunction. Characteristic skin manifestations, such as edema, erythema, induration, peau d'orange appearance, and the groove sign, are of diagnostic significance and observed in the majority of patients with EF. We herein report a case of EF without these characteristic skin manifestations. A 66-year-old Japanese woman developed progressive limb pain and motor dysfunction. No skin changes were observed. We diagnosed the patient with EF based on the clinical course, magnetic resonance imaging, and en bloc biopsy containing fascia and muscle. Oral prednisolone therapy markedly attenuated limb pain and motor dysfunctions. Through a systemic search of the medical literature, we retrieved 4 juvenile cases and 8 adult cases of EF without characteristic skin manifestations during the clinical course. We herein present a systemic review on EF without skin manifestations and discuss differences between the two proposed sets of diagnostic criteria of EF.

Rationale: Eosinophilic fasciitis (EF) is an uncommon connective tissue disorder characterized by limb and trunk erythema, with symmetrical thickening of the skin. Its pathogenesis is poorly understood. Treatment consists mainly of glucocorticoids. Yet, no randomized trials have evaluated therapies for this rare disease and the optimal treatment modality remains unclear. Although most patients show partial or complete response to glucocorticoids, many relapse upon drug tapering, while others either do not respond at all or fail to sustain prolonged remission. Second-line therapy for this rare disorder includes mainly methotrexate (MTX), azathioprine, cyclosporine and hydroxychloroquine. Recently, several attempts using rituximab and intravenous immunoglobulins (IVIG) have shown good clinical results., Patient Concerns: The three patients had good clinical response to glucocorticoid treatment, followed by disease flare when the drug dose was tapered. Adding methotrexate in all patients and azathioprine to patient 3 did not lead to remission., Diagnoses: EF was diagnosed in all patients based on clinical presentation accompanied by fascia biopsy that demonstrated eosinophilic fasciitis., Interventions: The patients were successfully treated with rituximab or IVIG, achieving sustained remission., Outcomes: The three cases had good clinical response to glucocorticoid treatment, followed by disease flare when the drug dose was tapered. The patients were then successfully treated with rituximab or IVIG, achieving sustained remission., Lessons: This review of three cases of EF supports the results of previous reports, suggesting addition of rituximab and IVIG is an effective treatment for patients with refractory disease., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.)

Background: Shulman's disease, or eosinophilic fasciitis (EF), is a rare autoimmune disease, characterized by sclerodermic skin lesions with progressive induration and thickening of the soft tissues. Chronic graft-versus-host-disease (GVHD) presenting as EF is a very rare manifestation of cutaneous GVHD., Case Presentation: We report an unusual case of EF in a 46-year-old Caucasian male patient who had received an allogenic hematopoietic stem cell transplantation in the context of relapsed/refractory multiple myeloma. The diagnosis was challenging, with the patient presenting hepatic dysfunction, normal eosinophils count, and incomplete clinical signs. Magnetic resonance imaging (MRI) and skin biopsy confirmed the diagnosis of EF. Early initiation of specific treatment with corticosteroids and prednisolone achieved complete response., Conclusion: In practice, incomplete signs in this rare complication should lead to MRI as it is a major tool to guide decision-making based on the skin biopsy, allowing a rapid diagnosis and the initiation of treatment without delay.

Bilateral lower limb swelling is a common clinical scenario with a wide differential diagnosis. We present a young man with gradually worsening bilateral leg swelling, who was diagnosed with eosinophilic fasciitis. A 20 year old Hispanic male presented with a six week history of bilateral lower limb pain and swelling, later involving the upper limbs, but sparing the hands, feet and face. He had initial pitting, followed by non-pitting oedema and had a positive 'groove sign'. With peripheral eosinophilia, a clinical diagnosis of eosinophilic fasciitis was suspected and was later confirmed on histology. He improved with prednisolone initially and is currently maintained on tapering prednisolone alongside methotrexate.

Eosinophilic fasciitis is a relatively rare cutaneous fibrotic condition affecting the deep fascia of the extremities, with or without peripheral blood eosinophilia. To examine the characteristics of Japanese patients with eosinophilic fasciitis, we conducted a brief, multicenter, retrospective survey at seven university hospitals. In total, 31 patients were identified as having eosinophilic fasciitis, among whom 30 patients fulfilled the Japanese diagnostic criteria. The male : female ratio was 2.3:1, and the mean age was 47.7 years. Three of the patients were under 20 years old. The possible triggering factors included muscle training, sports, walking or sitting for a long time, physical work, insect bite and drug. Co-occurrence of morphea was observed in nine cases (29%), and malignancies were associated in three (two hematological malignancies and one internal malignancy). Immunological abnormalities in the serum showed positive antinuclear antibody, positive rheumatoid factor, increased aldolase levels and increased immunoglobulin G levels. The patients were treated with either monotherapy or combination therapy by oral prednisolone (20-80 mg/day), methotrexate (6-10 mg/week), cyclosporin (100-150 mg/day), mizoribine, infliximab and phototherapy. Methylprednisolone pulse therapy was performed in six cases. By contrast, spontaneous improvement due to resting only was observed in two cases, and skin hardening was improved by withdrawal of the anticancer drug in one case. This study suggests several characteristics of Japanese patients with eosinophilic fasciitis, namely male predominance, rare pediatric occurrence, immunological abnormalities and coexistence with morphea. Systemic prednisolone is the first-line therapy, but pulse therapy is occasionally required for severe cases. The triggering events of physical stress are not so frequent as have previously been reported, and various factors or even unknown factors may be associated with the induction of eosinophilic fasciitis., (© 2020 Japanese Dermatological Association.)

Heel pain or achillodynia is one of the most common manifestations in patients with rheumatic inflammatory diseases (RID) and particularly spondyloarthritis (SpA). It can be associated with inflammation at the bone insertion of tendon, ligament, bursa or fascia. However, treatment is still a challenge for rheumatologists. Several findings highlighted the proven benefit of nonsteroidal anti-inflammatory drugs (NSAIDs), disease-modifying antirheumatic drugs (DMARDs), and recently, tumor necrosis factor (TNF)-α inhibitors. However, only limited data about the efficacy of local therapy such as glucocorticoid and anti-TNF injections are available. The aim of this systematic review was to assess the efficacy and safety of local therapies in heel pain and to make recommendations for further studies. Five studies discussing the effectiveness of local treatments of heel pain in RID were included. All studies recognized that the ultrasonography (US)-guided local corticosteroid or etanercept injections were effective and safe modalities for the treatment of inflammatory heel enthesitis, tendinitis, and retrocalcaneal bursitis (RCB) in patients with RID. Pain relief at the local site was associated with a reversion of the acute inflammatory changes in the heel. Furthermore, US-guided injection in RCB with a lateral approach was beneficial in terms of preventing side effects.

Eosinophilic fasciitis is a rare disease from the group of scleroderma-like connective tissue diseases with unclear etiopathogenesis. It may be occasionally accompanied with other eosinophilic or autoimmune dysfunctions (1,2). Lack of international diagnostic criteria and treatment consensus may lead to diagnostic and therapeutic difficulties. The 61-year-old man with no significant personal or family pathological history was admitted to the Dermatology Department presenting persistent induration for several months as well as erythema and pain of the shins that gradually extended to thighs and forearms, with limited mobility of peripheral joints. (Figure 1) Additional tests showed leukocytosis with 16% eosinophilia, elevated CRP, and hypergammaglobulinemia. Borrelia burgdorferi antibodies (classes IgM and IgG) were negative twice. A biopsy that included deep fascia was taken for histopathological examination. Antinuclear antibody screening was negative, but the direct immunofluorescence showed complexes in the dermo-epidermal junction and around the vessels. The diagnostics conducted toward malignant process showed no disturbing abnormalities (i.e. tumor markers in serum, chest, and abdomen computed tomography imaging, panendoscopy). The treatment was carried out with cephalosporin and nonsteroidal anti-inflammatory drugs (NSAIDs). The condition did not improve much but was stable. Histopathological results were indicative of eosinophilic fasciitis with fibrous thickening of deep fascia and perivascular infiltrations of plasma cells and lymphocytes; oral prednisone was initiated and the condition begin to improve. After 12 weeks, we observed disease progression with fever and very hard and cyanic skin lesions, which presented as an orange peel with linear furrows over the superficial venous vessels (Figure 2). The lesions extended to the trunk and caused troubles in moving. A complex rehabilitative intervention was started to minimize the inflammatory fascial restrictions. The prednisolone dose was increased, and oral methotrexate was added. After two weeks, the patient suffered from abdominal pain and periodic bleeding diarrhea. Methotrexate was suspected of inducing gastrointestinal adverse effects, and antipyretic NSAIDs were completely withdrawn. Colonoscopy showed features of mucosal edema with erythema, and histopathological examination revealed eosinophilic colitis. The patient was referred to a gastroenterologist, and methotrexate was ceased and switched to azathioprine. In summary, the consensus therapy of the rheumatologist, dermatologist, and gastroenterologist consisted of prednisolone and azathioprine. As of this writing, the patient's condition is gradually improving. The most characteristic symptoms of eosinophilic fasciitis is sudden onset with induration, sclerosis, and pain of the skin, with subcutaneous tissue and fascia usually appearing on the upper and lower limbs (3,4). The skin surface forms a characteristic orange peel appearance. The "groove" sign refers to the linear furrows over the superficial vessels of the extremities (1). Typical abnormalities are eosinophilia, elevated CRP, and hypergammaglobulinemia. The presence of eosinophilia is the most characteristic feature, occurring in 60-93% cases, but it is not necessary for diagnosis (1,5). Antinuclear antibodies are commonly absent with positive lesional direct immunofluorescence (6). If antinuclear antibodies are positive, it is recommended to broaden the diagnostic process to include other connective tissue diseases. Eosinophilia must be differentiated from hematological disorders and paraneoplastic syndrome. (4,6). Eosinophilic colitis is an eosinophilic gastrointestinal disease (EGID). It is the least frequent manifestation of EGID. It may be associated with connective tissue diseases, mostly systemic sclerosis - to our knowledge, there is no information in the literature about coexisting eosinophilic fasciitis. (7,8). The case described herein demonstrated that such a connection may occur. In treatment, it is important to prevent the patient from contractures and to maintain joint mobility by appropriate physiotherapy (2,9). The fascia forms a functional integral and continuous structure. Inflammation of one part of it changes the elasticity of the whole and produces fascial restrictions with movement limitation and pain. The fascia is profusely innervated, which favors constriction as a result of inflammation, and is also poorly vascularized which disrupts its regeneration (9,10). Myofascial techniques improve fascia elasticity by breaking up the tissue adhesions caused by inflammation (11). Eosinophilic fasciitis is a rare clinical entity, but knowing the possible clinical symptoms and laboratory abnormalities should help in taking the appropriate diagnostic path. It is important to treat the patient with attention to all concomitant diseases in consultation with different specialists.

Eosinophilic fasciitis (EF) is a rare condition in children that is typically treated with systemic corticosteroids. We present the case of a 9-year-old boy with biopsy-proven EF, refractory to systemic corticosteroids and methotrexate. The tyrosine kinase inhibitor imatinib was added as adjuvant therapy, leading to improvement in joint function and skin laxity. Our case is the first to suggest the anti-fibrotic properties of imatinib may benefit EF patients., (© 2020 Wiley Periodicals LLC.)

Background: This systematic review aims to evaluate the effectiveness and safety of silver acupuncture in treatment of myofascitis., Methods: Electronic databases of all silver acupuncture for myofascitis will be searched at PubMed, Cochrane Library, Springer, Embase, China National Knowledge Infrastructure, Wanfang, and Chinese Biological Medical disc from inception to March 31, 2020, with language restricted in Chinese and English. The primary outcome is visual analog scale, a short pain scale with sensitivity and comparability. Secondary outcomes included Clinical Assessment Scale for Cervical Spondylosis, Japanese Orthopaedic Association Scores, Oswestry dysfunction index, American Orthopaedic Foot and Ankle Society-Ankle Hindfoot scale, Foot and Ankle Ability Measure, The Cumberland ankle instability tool, Pittsburgh sleep quality index, self-rating anxiety scale, self-depression rating scale, and follow-up relapse rate. The systematic review and searches for randomized controlled trials of this therapy for myofascitis. The Cochrane RevMan V5.3 bias assessment tool is implemented to assess bias risk, data integration risk, meta-analysis risk, and subgroup analysis risk (if conditions are met). Mean difference, standard mean deviation, and binary data will be used to represent continuous results., Results: This study will provide a comprehensive review and evaluation of the available evidence for the treatment of myofascitis with this therapy., Conclusion: This study will provide new evidence to evaluate the effectiveness and side effects of silver acupuncture for myofascitis. Due to the data are not personalized, no formal ethical approval is required., Ethics and Dissemination: There is no requirement of ethical approval and it will be in print or disseminated by electronic copies., Prospero Registration Number: CRD42020151476.