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.

UNASSIGNED: Eosinophilic Fasciitis (EF) is a rare disease, originally proposed as \"diffuse fasciitis with eosinophilia\" by Shulman in 1974. Symptoms of EF include peripheral eosinophilia accompanied by symmetrical inflammation of the subcutaneous fascia and muscle, usually locating in the upper arms or thighs. There is no approved standard of care treatment.
UNASSIGNED: Taking into account that eosinophils may be pathogenetically involved in EF, we performed a review on Medline focusing on anti-Interleukin-5 (IL-5) therapies in EF.
UNASSIGNED: Only one case of a patient with EF has been reported who was successfully treated with reslizumab, an anti-IL-5 therapy. The patient had EF refractory to the commonly used immunosuppressive treatment but when reslizumab was added, the patient experienced remission of her symptoms.
UNASSIGNED: The exact aetiology of EF is still unclear, and many therapeutic approaches have been tested. Commonly used immunosuppressive agents, such as corticosteroids are not always effective and associate with significant side effects. Eosinophils seem to have a role in the pathogenesis of the disease; anti-eosinophilic therapies targeting IL-5/IL-5 Receptor could be an attractive alternative for the treatment of the disease.

Shulman\'s disease (eosinophilic fasciitis) is a very rare autoimmune disorder with an unknown etiopathogenesis. During the initial period of disease, it usually causes limb and trunk edema followed by collagenous thickening of the subcutaneous fascia. Eosinophilia is a predominant laboratory finding during the initial phase of the disease and less prominent in the later phases. Patients may also present with arthritis, myositis, peripheral neuropathy, and rarely pleuropericarditis. Here, we are reporting a case of eosinophilic fasciitis presenting with vague constitutional symptoms, fever, and peripheral blood eosinophilia followed by rapidly evolving skin tightening with joint contractures and muscle stiffness, which misled the treating team toward Scleroderma and its overlap syndromes. The diagnosis was finally clinched by a full-thickness skin biopsy along with underlying fascia and muscle tissue from an effected area, with a gratifying treatment response to standard immune suppression.

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.
UNASSIGNED: Die eosinophile Fasziitis (EF, auch Shulman-Syndrom) ist eine seltene Erkrankung des Bindegewebes mit entzündlicher Verdickung der Faszien sowie Schwellung und Verhärtung der Haut. Betroffen sind v. a. die distalen Extremitäten. Typische klinische Befunde stellen eine lokalisierte Schwellung und Verhärtung der Haut sowie das Groove-Sign (deutsch: Rillenzeichen/negatives Venenzeichen/Matratzenphänomen) dar. Der Goldstandard für die Diagnosesicherung ist bisher der bioptische Nachweis entzündlich verdickter Faszien. In der Literatur wird alternativ die Diagnosesicherung durch MRT-Bildgebung diskutiert. Wir berichten über einen Fall von asymmetrischer EF bei einem 54 Jahre alten, männlichen Deutschen. Die Vorstellung erfolgte mit schmerzhafter Verhärtung im Bereich des rechten Unterarms sowie charakteristischem Groove-Sign und einer Bewegungseinschränkung der rechten Hand. Im Blutbild zeigte sich eine Eosinophilie mit 0,57 G/l bzw. 9,6 % (norm. 0,05–0,5 G/l und 0,5–5,5 %), ANAs und ENAs waren negativ. Die Diagnosesicherung erfolgte histologisch, zusätzlich konnten wir verdickte Faszien sowohl im MRT als auch in der Sonographie nachweisen. Im Verlauf manifestierte sich die EF auch am linken Malleolus lateralis. Die Therapie erfolgte mit Prednisolon und Methotrexat.

The hallmark of eosinophilic fasciitis (EF) is painful symmetric skin stiffness and limb sclerosis with deep fascia inflammation. Two cases of unilateral EF have been reported in the literature but were not related to strenuous physical activity. In this report, a young female presenting with unilateral scleroderma-like skin changes, blood and tissue eosinophilia, and hypergammaglobulinemia proven to be EF after strenuous physical activity is described. This case represents the first case reported in the current literature of unilateral EF after vigorous exercise. A skin to muscle biopsy and magnetic resonance imaging (MRI) study indicated classic EF features. Cases of unilateral eosinophilic fasciitis are under-recognized and have rarely been described in the literature. EF requires a high index of suspicion to be diagnosed accurately and differentiated from another scleroderma variant, especially localized types. Early diagnosis and management of unilateral EF are very crucial because EF management and prognosis are completely different from other scleroderma mimics. EF heals rapidly in response to glucocorticoid or combination therapy. In the current case, corticosteroids alone failed to induce complete disease remission, but with the addition of methotrexate, complete resolution of both skin and systemic features was obtained. She is now in good health with no medication use. The current literature review indicates that this the first reported case of unilateral EF after vigorous exercise.

OBJECTIVE: 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.
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.

OBJECTIVE: First recognized in 1974, eosinophilic fasciitis (EF) is a fibrosing disorder of the fascia with characteristic cutaneous and hematologic manifestations. This review discusses recent trends in the diagnosis and treatment of EF.
RESULTS: Although fascial biopsy has classically been considered the gold standard for making a diagnosis of EF, radiologic imaging, particularly magnetic resonance imaging, has been increasingly used for both diagnosis and monitoring of treatment response. Systemic corticosteroids remain the first-line treatment for EF; however, their often prolonged use in the treatment of EF has prompted a search for adjunctive therapies. Methotrexate has emerged as the leading corticosteroid-sparing agent for EF. Since EF was initially described over 40 years ago, important diagnostic and therapeutic progress has been made. Future efforts should be directed at the pursuit of prospective studies including clinical trials and evidence-based guidelines.