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.

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: 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.

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.

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.

[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.

The Role of Electrodiagnostic Testing, Imaging, and Muscle Biopsy in the Investigation of Muscle Disease.

PURPOSE OF REVIEW: This article reviews the roles of electrodiagnostic testing, imaging studies (MRI and ultrasound), and muscle biopsy in evaluating patients for possible muscle diseases. RECENT FINDINGS: In addition to electrodiagnostic testing and muscle biopsy, muscle imaging is increasingly being used in the evaluation of patients with suspected muscle disease. MRI and ultrasound can help identify patterns of muscle involvement that may narrow the differential diagnosis and guide further testing. In addition, imaging can identify potential targets for muscle biopsy and can help evaluate for and exclude certain conditions that may mimic muscle disease. SUMMARY: This article provides a comprehensive overview of various testing modalities used in the evaluation of patients with suspected muscle disease, including electrodiagnostic studies, muscle imaging, and biopsy. In combination with a thorough history and clinical examination, these modalities can help narrow the differential diagnosis or, in certain cases, can confirm a specific etiology of muscle disease.

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.

Fascitis nodular en la región frontal: a propósito de un caso excepcional y revisión de la literatura / Nodular fasciitis of the forehead: presentation of an unusual case and a review of the literature

La fascitis nodular es una entidad excepcional en el territorio maxilofacial, consiste en una proliferación benigna del tejido fibroblástico, de etiología desconocida y con características clínicas e histopatológicas similares a los sarcomas. La clínica habitual consiste en una masa de consistencia dura, indolora y de rápido crecimiento. Su diagnóstico se realiza mediante una biopsia y un estudio histopatológico. El tratamiento consiste en la exéresis quirúrgica completa de la lesión. El diagnóstico diferencial es esencial dado el alto potencial de confusión diagnóstica con lesiones sarcomatosas debido a la similitud de sus características clínicas e histopatológicas. Se presenta el segundo caso clínico en la literatura de fascitis nodular en la región frontal, un varón de 43 años de edad, sin sufrir traumatismo previo en dicha localización. Se procedió a la biopsia-exéresis de la lesión con el diagnóstico histológico de fascitis nodular. Sin evidencia de recurrencia del tumor y un resultado estético excelente (AU)

Nodular fasciitis (NF) is a benign proliferation of fibroblast tissue of unknown etiology. It has clinical and histopathological characteristics similar to sarcomas, and is unusual in the maxillofacial area. The usual clinical presentation is a hard consistency, painless and fast growing mass of a hard consistency. Diagnosis is made by a biopsy and histopathology. NF treatment is complete surgical excision of the tumor. The differential diagnosis is essential for the high potential of confusion with sarcomatous lesions, due to the similarity of their clinical and histopathological features. The second case of nodular fasciitis in the frontal region to be reported in the literature is presented, a 43 year-old male, with no previous trauma in the region. Biopsy-excision of the mass was performed and the histological study gave the diagnosis of nodular fasciitis. The patient had no evidence of tumor recurrence and excellent aesthetic results were obtained (AU)

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.

Neuropsychological Correlates of Brain Perfusion SPECT in Patients with Macrophagic Myofasciitis.

BACKGROUND: Patients with aluminum hydroxide adjuvant-induced macrophagic myofasciitis (MMF) complain of arthromyalgias, chronic fatigue and cognitive deficits. This study aimed to characterize brain perfusion in these patients. METHODS: Brain perfusion SPECT was performed in 76 consecutive patients (aged 49±10 y) followed in the Garches-Necker-Mondor-Hendaye reference center for rare neuromuscular diseases. Images were acquired 30 min after intravenous injection of 925 MBq 99mTc-ethylcysteinate dimer (ECD) at rest. All patients also underwent a comprehensive battery of neuropsychological tests, within 1.3±5.5 mo from SPECT. Statistical parametric maps (SPM12) were obtained for each test using linear regressions between each performance score and brain perfusion, with adjustment for age, sex, socio-cultural level and time delay between brain SPECT and neuropsychological testing. RESULTS: SPM analysis revealed positive correlation between neuropsychological scores (mostly exploring executive functions) and brain perfusion in the posterior associative cortex, including cuneus/precuneus/occipital lingual areas, the periventricular white matter/corpus callosum, and the cerebellum, while negative correlation was found with amygdalo-hippocampal/entorhinal complexes. A positive correlation was also observed between brain perfusion and the posterior associative cortex when the time elapsed since last vaccine injection was investigated. CONCLUSIONS: Brain perfusion SPECT showed a pattern of cortical and subcortical changes in accordance with the MMF-associated cognitive disorder previously described. These results provide a neurobiological substrate for brain dysfunction in aluminum hydroxide adjuvant-induced MMF patients.

[The first report of a disseminated idiopathic myofasciitis in a ferret (Mustela putorius furo) from Germany]. / Erstbeschreibung einer disseminierten idiopathischen Myofasciitis bei einem Frettchen (Mustela putorius furo) aus Deutschland.

A 1.5 year old neutered male pet ferret (Mustela putorius furo) was presented with a sudden onset of severe weakness. The ferret was kept with three healthy mates, was vaccinated against distemper regularly and was never ill before presentation. Clinically, the ferret was depressed, had a hyperthermia of 40.3 degrees C, tachypnea and ocular as well as nasal discharge. Blood work revealed a mild neutropenia, blood chemistry a hyperglycemia, hyperbilirubinemia, hypoproteinemia, hypoalbuminemia, hypocalcemia and hyponatremia. Despite intensive therapy including fluid replacement, antibiosis, analgesia and antipyretics, the overall condition of the ferret deteriorated and the animal was euthanized two days later. Necropsy revealed a pyogranulomatous myositis, fasciitis and steatitis of the long hyoid muscles, the esophagus and intestine. Lesions were consistent with the disseminated idiopathic myositis of ferrets. This is the first reported case of this disease in a ferret originated in Germany.

[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.

Inflammation of the thoracolumbar fascia excites and sensitizes rat dorsal horn neurons.

BACKGROUND: Recent data show that the thoracolumbar fascia can be a source of pain. However, the spinal neuronal mechanisms underlying pain from a pathologically altered fascia are unknown. The present study aimed at finding out how dorsal horn neurons react to input from a chronically inflamed thoracolumbar fascia. METHODS: Recordings from rat dorsal horn neurons were made in the spinal segment L3. Twelve days before the recordings, the thoracolumbar fascia was inflamed by injection of complete Freund's adjuvant. Control animals received an injection of isotonic saline. In addition, behavioural experiments were carried out. RESULTS: Neurons in the spinal segment L3 do not normally receive input from the fascia, but 11.1% of the neurons did when the fascia was inflamed. Compared with control, the proportion of neurons having input from all deep somatic tissues rose from 10.8% to 33.3% (p < 0.02). Moreover, many neurons acquired new deep receptive fields, most of which were located in the hindlimb (p < 0.04). Surprisingly, the pressure pain threshold of the inflamed rats did not change, but they showed a reduction in exploratory activity. CONCLUSIONS: One of the prominent findings was the appearance of new receptive fields in deep tissues of the hindlimb. Together with the expansion of the spinal target region of fascia afferents into the segment L3, the appearance of new receptive fields is a possible explanation for the spread of pain in patients with non-specific low back pain.