Steroid-resistant eosinophilic fasciitis successfully treated with addition of extracorporeal photopheresis.

Eosinophilic fasciitis (EF) is an uncommon fibrosing disease of the fascia with characteristic cutaneous and hematologic manifestations. Although EF is most commonly treated with corticosteroids at the beginning, a considerable number of patients show an inadequate response and hence various therapeutic strategies have been tried, including extracorporeal photopheresis (ECP). We describe the case of a 66-year-old woman with steroid-resistant EF that improved significantly after ECP was added to her treatment regimen. To date, only six cases of this therapeutic strategy have been reported in English literature.

Intra-articular nodular fasciitis: a rare lesion case report and an updated review of the literature.

BACKGROUND: Nodular fasciitis is a benign proliferation of myofibroblasts that usually arises in subcutaneous tissues of the trunk, neck, head, and upper extremities of young adults. It is not reported to arise in the joints. CASE PRESENTATION: In this report, we describe a rare case where nodular fasciitis occurred in an intra-articular location in the right knee of a 20-year-old man. The patient presented with 3-months' duration of knee pain without history of trauma to the extremity. Physical examination revealed pain, joint effusion, and limited range of motion (ROM) of the affected knee. Magnetic resonance imaging (MRI) showed a 2.5 × 2 × 1 cm lesion in front of the posterior cruciate ligament. Arthroscopically, the soft tissue mass was removed and pathologically diagnosed as a rare, benign, intra-articular nodular fasciitis. Symptoms resolved 1 month after the operation and no recurrence was found at the 6 months follow-up. CONCLUSION: The present paper describes detailed characteristics of intra-articular nodular fasciitis and provides an updated comprehensive summary of 21 prior case reports.

Eosinophilic Fasciitis: A Single Center Experience of Seven Patients.

BACKGROUND: Eosinophilic fasciitis (EF) is a rare disease characterized by scleroderma-like skin, inflammation of deep muscle fascia, hypergammaglobulinemia, peripheral eosinophilia, and elevated erythrocyte sedimentation rate. OBJECTIVES: To present our experience in diagnosis and treatment of seven biopsy-proven EF patients in a large tertiary medical center. METHODS: We screened all patients who were admitted to our tertiary medical center and diagnosed with EF by tissue biopsies from January 2000 to January 2016. We analyzed relevant patient files regarding diagnosis, treatment, and outcome parameters. A comprehensive framework was presented based on the results of our observations and the corresponding literature. RESULTS: We identified seven patients (six males; one child). Mean age at diagnosis was 37.4 years (range 10-67 years). Underlying autoimmune disorders were observed in three patients (42.8 %). Disease anatomical distribution was noted in lower and upper limbs (85.7% and 57.1%, respectively) as well as neck and shoulders (14.3% each). Three patients (42.8%) had a history of initial misdiagnosis. The mean time period from first clinical presentation to histopathological diagnosis was 150.3 days (range 16-602 days). Treatment included oral glucocorticoids (71.4%), pulse methylprednisolone (14.2%), and methotrexate (42.8%). Recovery from symptoms related to EF was observed in six patients. CONCLUSIONS: Diagnosis of EF is primarily based on clinical and histopathological findings. As eradication of this disease can be expedited with early treatment, it is important to increase awareness in the medical community.

[Sarcoid-like granulomatosis associated with eosinophilic fasciitis]. / Fasciite de Shulman associée à un infiltrat granulomateux sarcoïdosique.

BACKGROUND: Eosinophilic fasciitis (EF) is a rare condition characterized by swelling of the extremities, sclerodermatous evolution and frequent hypereosinophilia. Hematological disorders, including aplastic anemia, solid tumors and autoimmune diseases, may be associated with EF. EF is usually not associated with granulomatous diseases. CASE REPORT: Herein we describe the case of an 80-year-old man with symmetrical swelling and sclerosis of the legs, typical of EF, associated with skin and lymph node granulomas. Oral prednisone treatment resulted in complete clinical remission. DISCUSSION: Association of EF and granulomatous disease is uncommon. Our case highlights the possible association of EF with sarcoidosis-like reactions.

Juvenile eosinophilic fasciitis: three case reports with review of the literature.

OBJECTIVES: Eosinophilic fasciitis is an uncommon scleroderma-like disorder characterised by induration and thickening of skin and soft tissue, usually associated with peripheral eosinophilia, poorly characterised in childhood. METHODS: We report 3 paediatric cases of eosinophilic fasciitis showing unusual clinical and histopathological features with a review of the literature. RESULTS: All cases presented progressive motility impairment started from upper limbs with no skin abnormalities. All cases showed systemic inflammatory involvement and 2 patients had acute complications. Two patients developed disabling outcomes despite appropriate treatments. CONCLUSIONS: Eosinophilic fasciitis may present unusual clinical and histopathological features during childhood and requires early recognition in order to prevent acute complications and disabling outcomes.

Finger stiffness or edema as presenting symptoms of eosinophilic fasciitis.

To investigate the clinical features and finger symptoms of eosinophilic fasciitis (EF), we reviewed five patients with EF. The chief complaint was pain, edema and/or stiffness of the extremities. The distal extremities were affected in all patients, and there was also proximal involvement in one patient. One patient had asymmetrical symptoms. All four patients with upper limb involvement had limited range of motion of the wrist joints, and three of them complained of finger symptoms. Two of these three patients showed slight non-pitting edema of the hands, and the other one had subcutaneous induration of the forearm. All four patients with lower limb symptoms had limited range of motion of the ankle joints, and two showed edema or induration of the legs. Inflammatory changes in the joints were not detected in any of the patients. Two patients displayed neither objective induration nor edema, and two patients had muscle tenderness. In conclusion, finger symptoms of patients with EF might be caused by fasciitis of the forearms, which leads to dysfunction of the long finger flexors and extensors as well as slight edema of hands. Limited range of motion of wrist and/or ankle joints indicates sensitively distal muscle dysfunction caused by fasciitis.

[Eosinophilic fasciitis]. / Eozynofilowe zapalenie powiezi.

Eosinophilic fasciitis is a rare connective tissue disease with unclear etiology and pathogenesis. It is classified as a scleroderma-like syndrome. The disease is characterized by fibrosis of the skin and subcutaneous tissues with significant thickening of fascia. Visceral involvement is rare. Characteristic feature in laboratory tests is peripheral blood eosinophilia. Differential diagnosis should be performed, including ruling out systemic sclerosis, nephrogenic systemic fibrosis, eosinophilia-myalgia syndrome, scleromyxedema, hypereosinophilic syndrome or Churg-Strauss syndrome. Final diagnosis is confirmed by histopathological examination. In treatment of the disease corticosteroids and/or immunosuppressive drugs are used. Some other drugs showed activity in this disease e.g. dapsone, infiximab or rituximab. Prognosis is rather good but sometimes a long-term treatment is necessary. In this paper we summarized the current knowledge on eosinophilic fasciitis.

Macrophagic myofasciitis: characterization and pathophysiology.

Aluminium oxyhydroxide (alum), a nanocrystalline compound forming agglomerates, has been used in vaccines for its immunological adjuvant effect since 1927. Alum is the most commonly used adjuvant in human and veterinary vaccines, but the mechanisms by which it stimulates immune responses remain incompletely understood. Although generally well tolerated, alum may occasionally cause disabling health problems in presumably susceptible individuals. A small proportion of vaccinated people present with delayed onset of diffuse myalgia, chronic fatigue and cognitive dysfunction, and exhibit very long-term persistence of alum-loaded macrophages at the site of previous intramuscular (i.m.) immunization, forming a granulomatous lesion called macrophagic myofasciitis (MMF). Clinical symptoms associated with MMF are paradigmatic of the recently delineated 'autoimmune/inflammatory syndrome induced by adjuvants' (ASIA). The stereotyped cognitive dysfunction is reminiscent of cognitive deficits described in foundry workers exposed to inhaled Al particles. Alum safety concerns will largely depend on whether the compound remains localized at the site of injection or diffuses and accumulates in distant organs. Animal experiments indicate that biopersistent nanomaterials taken up by monocyte-lineage cells in tissues, such as fluorescent alum surrogates, can first translocate to draining lymph nodes, and thereafter circulate in blood within phagocytes and reach the spleen, and, eventually, slowly accumulate in the brain.

Intra-articular nodular fasciitis of the knee: a rare cause of recurrent hemarthrosis.

A 20-year-old man presented with pain and recurrent hemarthrosis in the right knee. Magnetic resonance imaging of the knee showed a lesion with homogeneous low signal intensity on T1-weighted images and a heterogeneous, low to high signal intensity on T2-weighted images. At arthroscopy, the mass was located between the posterior cruciate ligament and the posterior knee joint capsule. The tumor was excised through a posterior approach and histologically diagnosed as a nodular fasciitis. Intra-articular nodular fasciitis is a very rare clinicopathologic entity. The current case showed the unique clinical feature of recurrent hemarthrosis at initial presentation, which has not been previously reported.

Infliximab may be effective in the treatment of steroid-resistant eosinophilic fasciitis: report of three cases.

OBJECTIVE: EF is a rare fibrosing disorder that may involve skin and internal organs. Current therapies include moderate- to high-dose glucocorticoids with or without use of immunosuppressives. METHODS: We report three cases of steroid-resistant EF in clinical practice who were treated with 3 mg/kg every 8 weeks infliximab therapy. RESULTS: All patients noticed an improvement in their symptoms, joint contractures and skin thickening within 8 weeks of starting infliximab therapy, ultimately leading to a drug-free remission (range 1-3 years). CONCLUSION: Based on this and other reported cases, infliximab may be beneficial in patients with steroid-resistant EF.

Proliferative fasciitis: A rare cause of disturbances in an adolescent hand.

We report a case of hand soft tissue tumor-proliferative fasciitis (PF)-in a 12-year-old patient that presented as a painful lump causing trigger finger. After meticulous diagnostic workup, a surgical excision led to immediate amelioration of symptoms. PF is a rare benign pseudosarcomatous lesion arising typically in the subcutaneous tissue and fascia in adults. It is very uncommon in the hand. To the best of our knowledge, this is the first report of a trigger finger being caused due to this pathology. In this report, the authors review PF lesions on hands, advice careful evaluation of magnetic resonance imaging features, and recommend surgical management.

Eosinophilic fasciitis: Current concepts.

Eosinophilic fasciitis (EF) is an uncommon connective tissue disease characterized by abrupt onset of edema, followed by progressive induration of primarily the distal extremities. Patients may exhibit inflammatory arthritis, joint contractures, decreased mobility, and nerve entrapment. Almost half of patients with EF may have coexisting morphea plaques. Classic laboratory studies display peripheral eosinophilia, hypergammaglobulinemia, and elevated inflammatory markers. EF is included in the spectrum of scleroderma like disorders and may be difficult to distinguish from other sclerosing skin disorders. Full-thickness biopsy containing muscle and fascia is considered the gold standard for diagnosis and reveals sclerosis of the middeep dermis, subcutaneous fat, and thickening of the fascia. Magnetic resonance imaging (MRI) has been increasingly utilized to augment diagnostic capabilities. Ultimately, the diagnosis of EF relies upon the combination of characteristic clinical, laboratory, imaging, and histologic findings. Although some patients experience spontaneous remission, systemic corticosteroids (SCS) are the mainstay of treatment. Patients who fail to improve with SCS alone require the addition of a second immunosuppressive drug. Additionally, although data are limited, there is evidence to suggest that initial combination therapy with SCS and methotrexate (MTX) may be most beneficial.

Cognitive dysfunction associated with aluminum hydroxide-induced macrophagic myofasciitis: A reappraisal of neuropsychological profile.

Patients with macrophagic myofasciitis (MMF) present with diffuse arthromyalgias, chronic fatigue, and cognitive disorder. Representative features of MMF-associated cognitive dysfunction include attentional dysfunction, dysexecutive syndrome, visual memory deficit and left ear extinction. Our study aims to reevaluate the neuropsychological profile of MMF. 105 unselected consecutive MMF patients were subjected to a neuropsychological battery of screen short term and long-term memory, executive functions, attentional abilities, instrumental functions and dichotic listening. From these results, patients were classified in four different groups: Subsymptomatic patients (n=41) with performance above pathological threshold (-1.65 SD) in all tests; Fronto-subcortical patients (n=31) who showed pathological results at executive functions and selective attention tests; Papezian patients (n=24) who showed pathological results in storage, recognition and consolidation functions for episodic verbal memory, in addition to fronto-subcortical dysfunction; and Extinction patients (n=9) who had a left ear extinction at dichotic listening test in association to fronto-subcortical and papezian dysfunction. In addition, inter-test analysis showed that patients with apparently normal cognitive functions (Subsymptomatic group) performed significantly worse to attention tests compared to others. In conclusion, our study shows that (i) most patients have specific cognitive deficits; (ii) all patients with cognitive deficit have impairment of executive functions and selective attention; (iii) patients without measurable cognitive deficits display significant weakness in attention; (iv) episodic memory impairment affects verbal, but not visual, memory; (v) none of the patients show an instrumental dysfunction.

Medial tibial stress syndrome (tibial fasciitis): a proposed pathomechanical model involving fascial traction.

Although medial tibial stress syndrome is one of the most common lower-extremity overuse injuries, its pathomechanics remain controversial. Two popular theories have been proposed to account for this condition: tibial bending and fascial traction. This article evaluates the role of fascial traction in medial tibial stress pathomechanics. We hypothesized that with contraction of the deep leg flexors tension would be imparted to the tibial fascial attachment at the medial tibial crest. We also speculated that circumferential straps would dampen tension directed to the medial tibial crest. The amount of strain present in the tibial fascia adjacent to its distal medial tibial crest insertion during loading of the leg was investigated as a descriptive laboratory pilot study using three fresh cadaver specimens. Strain in the distal tibial fascia was measured using strain gauges placed in the fascia at its medial tibial crest insertion. As tension on the posterior tibial, flexor digitorum longus, and soleus tendons increased, strain in the tibial fascia increased in a consistent linear manner (P < .0001). We conclude that fascial tension may play a role in the pathomechanics of medial tibial stress syndrome. The tenting effect of the posterior tibial, flexor digitorum longus, and soleus tendons caused by muscle contraction exerts a force on the distal tibial fascia that is directed to its tibial crest insertion. Circumferential straps provided no dampening effect on tension directed to the medial tibial crest.

[Functional assessment and rehabilitation of eosinophilic fasciitis]. / Intérêt du bilan fonctionnel et de la rééducation dans la fasciite à éosinophile.

PURPOSE: To evaluate functional assessment and rehabilitation in eosinophilic fasciitis. METHOD: Description of a clinical case of eosinophilic fasciitis, including the evolution of functional assessment after treatment with corticotherapy and rehabilitation. CASE REPORT: Our case was a 33-year-old patient with major walking disability and pain in the left calf. Biological examination and imaging, including nuclear magnetic resonance and bone scintigraphy, showed inflammation of the adipose and muscular tissues of the posterior area of the leg. Cutaneomuscular biopsy confirmed the diagnosis of eosinophilic fasciitis. Corticotherapy was then instituted in parallel with rehabilitation to limit deficiencies and disability function. DISCUSSION-CONCLUSION: Functional assessment used as indicating objective could, in partnership with follow-up of the biological inflammatory syndrome, lead to decreased use of corticoids in iosinophilic fasciitis. Moreover, supplemental rehabilitation could limit deficiencies and the functional disability resulting from the fasciitis.